Summary Introduction: Cystic dilatation of the bile ducts is a rare birth defect. Its incidence is of the order of 1/100,000 to 1/150,000 births. Surgery is indicated to avoid liver damage secondary to stasis and infection (hepatic cirrhosis). Observation: This was a 16-year-old patient seen in a surgical consultation for pain in the right hypochondrium, nausea, vomiting, subjaundice, and intermittent fever. The clinical history of the disease found a history of 2 poorly documented surgeries for the same pathology in 2016 and 2020. On examination, she was in good general condition, a subicterus, the abdomen is supple with no palpable mass. An abdominal CT scan concluded to a cystic dilation of the common bile duct. A total surgical resection of the cystic wall was performed followed by a hepatico-jejunal anastomosis in Y. The postoperative course is simple. Conclusion: Cystic dilation of the common bile duct is often difficult to diagnose. Its main progressive risks are lithiasis, cancerization and infection. These complications can be fatal and are prevented by early resection of the cyst, the only valid treatment. Keywords: Common bile duct cyst, Malignant degeneration, Children
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