Autosomal recessive polycystic kidney disease (ARPKD) affects the liver and the kidney. Renal involvement presents early in life, whereas hepatic involvement manifests slightly later with portal hypertension. A male toddler came with chronic abdominal distension, prominent abdominal wall vessels, and umbilical protuberance. Ultrasonography findings of hepatic fibrosis with portal hypertension, mildly prominent biliary radicals, bilateral cystic renal enlargement, and a striated nephrogram on contrast-enhanced computed tomography suggested the diagnosis. Congenital hepatic fibrosis is invariably associated with ARPKD leading to portal hypertension and the development of portosystemic collaterals; those located in the umbilical region appear as caput medusae.