Choledochal cyst is a rare disease of the biliary tract. Historically, choledochal cyst disease was considered as a disease of childhood but is increasingly being recognised in adults. The optimal and definitive treatment used today is the complete excision of the extrahepatic duct, cholecystectomy and roux-en-Y hepaticojejunostomy due to high morbidity and high risk for carcinoma associated with internal drainage, a commonly used treatment in the past. We report a case of 14 year old male patient with markedly dilated tortuous CBD with marked splenomegaly and portal hypertension. The treatment of Choledochal cyst complicated by portal hypertension has evolved from internal drainage of cyst to single stage excision of cyst with bilioenteric anastomosis. Portal decompression is reserved for cases with extensive collaterals
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