The complications of choledochal cyst are avoidable if diagnosed early, and adequate resection undertaken. This case report describes the long history of right subcostal pain in a young man of 26 who had a squamous carcinoma in a choledochal cyst diagnosed after serial section of the excised cyst. Subsequent resection of the head of the pancreas showed histological residual tumour from which he died 4 months later. This case illustrates the need for complete early excision of a choledochal cyst to prevent this complication.
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