Introduction: Choledochal cysts in adults are commonly associated with hepatobiliary pathology and complications of previous cyst related procedures. Portal hypertension is a rare complication of choledochal cyst. The treatment of choledochal cyst complicated by portal hypertension has evolved from internal drainage of cysts to single stage excision of cyst with bilioenteric anastomosis. Case Report: A 15yearold female presented with typical triad of abdominal pain, abdominal lump and jaundice. Magnetic resonance cholangiopan creatography (MRCP) was suggestive of typeI choledochal cyst with portal hypertension. An upper gastrointestinal endoscopy revealed grade 1 esophageal varices with proximal gastropathy. Intraoperatively, the posterior wall of the choledochal cyst was densely adherent to the portal vein and hence a partial excision of cyst with stripping of the mucosa of the posterior wall of the cyst along with RouxenY hepaticojejunostomy was done. Conclusion: Single stage excision of choledochal cyst with bilioenteric anastomosis is the treatment of choice of choledochal cyst with portal hypertension. Portal decompression is reserved for cases with extensive collaterals in the hepatoduodenal ligament.
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