The adult polycystic kidney disease(APCK) is inheritant disease in which cysts aredistributed throught the cortex and medulla of both kidneys. The disease process is usually notclinically apparent until the third or fourh decade of life. Mild persistent proteinuria(> 200mg/d) can be developed in 20% to 40% of cases, but nephrotic-range proteinuria is rare and whenfound, should prompt a search for the other concomitant renal disorder, such as IgA nephropathy, rapidly progressive glomerulonephropathy, focal glomerulosclerosis.

We report a case of APCK presented with nephrotic syndrome. A 76-year-old man was admitted due to generalized edema with weakness. The blood pressure was120/80mmHg. The BUN/creatinine were 15/1.2mg/dl, serum albumin 2.9g/dl, cholesterol / triglyceride 432/273mg/dl, total urinary protein excretion 8.41g/day.

The serum level of IgG, IgAS, IgM, C₃, C₄ were within normal range and anti-nuclear antibody and anti-double strand DNA antibody were also negative. The abdominal ultrasonography and computerized tomography revealed multiple large cysts in both kidneys.The patient was treated with Enalapril®, an angiotensin converting enzyme inhibitors, 5mg forcontrol of proteinuria.

The APCK presented with nephrotic syndrome is rare and to our knowledge,this is a first report in Korea.