Nephropathy related to primary coenzyme Q 10 (CoQ10) deficiency is a rare inherited disease. COQ8B (also known as ADCK4)-associated nephropathy has been reported in a Korean cohort with six patients accompanied by medullary nephrocalcinosis.  In here, we report a novel pediatric case with COQ8B mutation with medullary nephrocalcinosis and present the results from a systematic review on COQ8B-associated nephropathy. Electronic databases were searched using related terms (~Feb 2nd, 2020).  A 4-year-old girl presented with isolated proteinuria, normal serum lab, and medullary nephrocalcinosis. We performed a direct Sanger sequencing for COQ8B, which found her with compound heterozygous mutations (c.737G>A, exon 9; c.1507C>T, exon 15). A kidney biopsy showed focal segmental glomerulosclerosis and abnormally increased mitochondrial accumulation was found in the podocytes cytoplasm. After 8 weeks of CoQ10 supplement and 4 weeks of cyclosporine treatment, her urine protein to creatinine ratio decreased to 0.5 mg/mg. From 127 articles searched, there were 11 eligible studies with 48 patients with COQ8B-nephropathy. Male to female ratio was 1:2 and median age at diagnosis was 14.6 years. Major histology was FSGS (32/48, 67%) and mitochondrial aggregation in the podocytes were found in 7 (14%) patients. Patients had chronic kidney disease in 44% and progressed to end-stage renal disease in 25%. There were six transplantation cases with none recurred. Medullary nephrocalcinosis was reported only in 7 Korean patients. Of the 14 patients supplemented with CoQ10, 7 reported improved outcomes. Calcineurin inhibitor was tried in 7 patients which induced partial remission in 4 cases. COQ8B-associated FSGS is a rare hereditary nephropathy which can benefit from early diagnosis and CoQ10 supplement. So far, patients with COQ8B mutation reported in South Korea had medullary nephrocalcinosis. Although the disease is not immune-mediated, calcineurin inhibitor appears efficacious. Early suspicion based on this phenotype may improve the prognosis.