The clinical findings suggest autosomal recessive polycystic kidney disease. The diagnosis of autosomal recessive polycystic kidney disease is strongly suggested by bilateral palpable flank masses in an infant with pulmonary hypoplasia, oligohydramnios and hypertension and the absence of renal cysts in the parents. On imaging and biopsy the kidney shows innumerable cysts radiating from medulla to the cortex. Autosomal recessive kidney disease is associated with liver disease in about 45% cases. Both kidneys are markedly enlarged and grossly show innumerable cysts throughout the cortex and medulla. Microscopic studies demonstrate micro cysts radiating from medulla to the cortex located primarily within the collecting tubules and the ducts. Development of progressive interstitial fibrosis and tubular atrophy during advanced stages of disease eventually leads to renal failure. Liver involvement is characterized by bile duct proliferation and ectasia as well as by hepatic fibrosis