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Background: Complete cyst excision of the extrahepatic disease component with biliary reconstruction on proximal healthy bile ducts is considered to be the treatment of choice in patients with congenital bile duct cysts (BDC). Proximal cystic disease that extends to the roof of the main biliary convergence (MBC) might challenge this standard of surgical care. Methods: A retrospective multicenter study was conducted in 4 European surgical centers concerning their experience with adult patients suffering from type I and IV BDC according to the Todani classification. Clinical presentation, operative management, and postoperative outcome were compared between patients with or without proximal extrahepatic cystic disease that involved at least the roof of the MBC (defined as being BDC with MBC involvement subgroup). Results: From an overall series of 49 adult patients suffering from type I or IV BDC according to the Todani classification, 7 patients had BDC with MBC involvement (14%). Patient age, clinical presentation, duration of symptoms, associated major coexistent hepatobiliary and pancreatic diseases, and synchronous cancer were not significantly different in these patients compared with a control group of 42 adult patients with BDC without MBC involvement. Incomplete proximal cyst excision rate was 86% in the cases of BDC with MBC involvement. Early and late postoperative results were similar in BDC with MBC involvement and in the control group of adult patients, but the incidence of subsequent cancer was significantly higher in the BDC with MBC involvement group (29% vs 0%; P < .02). Conclusion: BDC that involves the roof of the MBC is a real surgical challenge to obtain complete proximal cystic disease excision. As suggested in this small study, primary incomplete excision of this particular form of BDC might expose the patient to the risk of subsequent cancer, a feature that must be confirmed in larger series. [Copyright &y& Elsevier]