Autosomal recessive polycystic disease is a rare hepatorenal disorder. End-stage renal disease and liver fibrosis are serious presentations of this disease. Here, we report 2 brothers with autosomal recessive polycystic disease who presented with abnormal abdominal protrusion and hepatosplenomegaly during infancy and eventually underwent renal transplant. Congenital hepatic fibrosis and nephromegaly followed by renal failure developed, after which renal transplant was successfully performed. The remaining compli-cation after transplant was hematemesis and melena due to esophageal varices. Autosomal recessive polycystic disease has a broad spectrum of symptoms; similar pre-sentations with manifestations in siblings may explain some unknown genetic causes of this rare disease.