Abstract: Caroli disease is a non-obstructive dilatation of the intrahepatic bile ducts. There are two forms of Caroli's disease, one associated with congenital hepatic fibrosis and a simpler form occurring alone. The latter known as Caroli's disease may be associated with autosomal recessive polycystic kidney disease.

Case Report: A 5 year old boy born of non-consanguineous marriage presented with fever, epistaxis and breathlessness 10 days back for which he was admitted in a private hospital and treated with intravenous antibiotics and blood transfusion. He was referred for further management. On examination he was in respiratory distress with hypertension, was malnourished, had jaundice, pallor and generalized edema. Systemic examination revealed hepatosplenomegaly with ascitis. Other investigations showed anemia with hyperkalemia and elevated blood urea nitrogen and serum creatinine. He had hypocalcemia with hyperphosphatemia with normal alkaline phosphatase. Venous blood gas showed metabolic acidosis. His liver function tests were normal. Ultrasound of abdomen showed multiple cysts in the liver with bilateral hydronephrosis and hydroureter with splenomegaly and multiple collaterals. CT abdomen showed central intrahepatic cysts with dilated biliary radicles with bilateral polycystic kidneys (Figure 1). Parents ultrasound of kidneys was normal. He was thus diagnosed as a Caroli's disease. He was treated with bicarbonate infusion, calcium supplements, blood transfusion and antihypertensives with potassium binders. He was advised dialysis and renal transplant, which

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