We reported two cases of glomerulocystic kidney (GCK). Case 1 was a 35-day-old male who was born at 38 weeks of gestation and weighed 1750 g at birth, He showed chromosomal aberration and surface anomaly, and was dia-gnosed as having pentalogy of Fallot. There were no signs of renal disturbance. At autopsy, subcapsular cysts composed of dilated Bowman's capsules were found. Case 2 was a 63-year-old man. He suffered from mild cerebral infarction at the age of 45. At the age of 51, he was diagnosed as having chronic glomerulonephritis and began hemodialysis at the age of 55. He died of uremic lung at 63. Diffuse dilatation of Bowman's capsules were found in the renal cortices. In the liver, proliferation and cystic dilatation of interlobular bile ducts were noted. The patients with GCK without any severe disease in other organs show no or stable symptoms in the early period of life, but later developed severe renal insufficiency.