Objective: First, we generated patient specific hiPSC lines (CMC-Fb01,02) using peripheral blood mononuclear cells from patients who were diagnosed as fabry disease by GLA gene sequencing. Second, we generated GLA-KO hiPSCs using wild-type hiPSC (WTC-11) by CRISPR/Cas9 technology. Lastly, using all of wild type, patient specific (CMC-Fb01, 02) and disease specific hiPSC lines (GLA-KO-WTC-11), we generated kidney organoids and compared alpha-galactosidase A (α-GLA) activity, globotriaosylceremide (GB-3)(LC_MS)and also GB-3 deposition using immunofluorescent (IF) staining and also zebra body formation under electromicroscopy (EM) among kidney organoids derived from 3 types of hiPSCs. Methods: Patient specific hiPSCs contained same frame shift mutation in GLA genes which was detected in PBMCs of their originating patients and both hiPSCs lines showed typical pluripotency markers and also normal karyotyping. Kidney organoids generated from 3 types of hiPSC lines showed typical nephron markers such as PODXL, LTL, and E-cad in IF staining. In both kidney organoids derived from patients specific hiPSC and disease specific hiPSCs, α-GLA activity was significantly decreased in comparison with wild type kidney organoids. In contrast, GB3 level measured by LC-MS and also GB-3 deposits by IF staining was significantly increased in the patient specific and also disease specific kidney organoids in comparison with wild-type kidney organoids. In EM finding, typical zebra body was detected in both patient specific and disease specific kidney organoids. Results: Kidney organoids generated using hiPSCs derived from fabry disease patients may recapitulate the phenotype of fabry disease nephropathy, and it may be used as a potential platform for an in vitro disease modeling.   Conclusions: Objective: The aim of this study is to explore the possibility of human induced pluripotent stem cells (hiPSC) -derived kidney organoids for fabry disease modeling using both patient derived iPSC and also genetically modified (GLA-KO) hiPSC. Methods: First, we generated patient specific hiPSC lines (CMC-Fb01,02) using peripheral blood mononuclear cells from patients who were diagnosed as fabry disease by GLA gene sequencing. Second, we generated GLA-KO hiPSCs using wild-type hiPSC (WTC-11) by CRISPR/Cas9 technology. Lastly, using all of wild type, patient specific (CMC-Fb01, 02) and disease specific hiPSC lines (GLA-KO-WTC-11), we generated kidney organoids and compared alpha-galactosidase A (α-GLA) activity, globotriaosylceremide (GB-3)(LC_MS)and also GB-3 deposition using immunofluorescent (IF) staining and also zebra body formation under electromicroscopy (EM) among kidney organoids derived from 3 types of hiPSCs. Results: Patient specific hiPSCs contained same frame shift mutation in GLA genes which was detected in PBMCs of their originating patients and both hiPSCs lines showed typical pluripotency markers and also normal karyotyping. Kidney organoids generated from 3 types of hiPSC lines showed typical nephron markers such as PODXL, LTL, and E-cad in IF staining. In both kidney organoids derived from patients specific hiPSC and disease specific hiPSCs, α-GLA activity was significantly decreased in comparison with wild type kidney organoids. In contrast, GB3 level measured by LC-MS and also GB-3 deposits by IF staining was significantly increased in the patient specific and also disease specific kidney organoids in comparison with wild-type kidney organoids. In EM finding, typical zebra body was detected in both patient specific and disease specific kidney organoids. Conclusions: Kidney organoids generated using hiPSCs derived from fabry disease patients may recapitulate the phenotype of fabry disease nephropathy, and it may be used as a potential platform for an in vitro disease modeling.