Polycystic kidney disease (PKD) is characterized by the development of fluid-filled renal cysts, causing progressive kidney function decline, culminating in the need for renal replacement therapy or kidney transplant. PKD1 and PKD2, encoding polycystin (PC) 1 and 2, are the two major genes associated with ADPKD; other genes include IFT140, GANAB, DNAJB11, and ALG9. We performed next generation sequencing (NGS) in a 15-year-old girl affected by intermittent microscopic hematuria and tiny renal cysts progressed to multiple cysts An 8-year-old girl who had shown intermittent microscopic hematuria were referred to our hospital. Her prenatal ultrasound findings were normal. Laboratory findings showed normal serum chemical battery and no proteinuria. Initial kidney sonographic findings showed 0.3cm hyperechoic lesion with twinkling artifact at right kidney lower polar area, which were not changed thereafter. During seven years, she had shown intermittent microscopic hematuria only, and there are no family history of renal cystic diseases. Recently, we exam MRI and find multiple benign cysts in both kidneys. Therefore, we performed NGS and found a mono-allelic frameshift variant (c.282dup (p.Gln95ThrfsTer10)) in ALG5. Other genes including PKD1, PKD2, UMOD, GANAB, and DNAJB11 were not found. ALG5 gene, encoding asparagine-linked glycosylation protein 5 homolog (ALG5), is required for polycystin-1 (PC1) maturation and membrane and ciliary localization and that heterozygous loss of ALG5 have been shown to disrupt PC1 maturation and trafficking via under-glycosylation, causing an autosomal dominant polycystic kidney disease-like (ADPKD-like) phenotype (PKD7). We performed next generation sequencing (NGS) in a 15-year-old girl affected by ADPKD-like symptoms, such as microscopic hematuria and multiple renal cysts and identified a mono-allelic frameshift variant (c.282dup (p.Gln95ThrfsTer10)) in ALG5, which would be first report in Korea as far as we know