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The cystic dilatation of the common bile duct is a rare disease in Europe and in the USA; even rarer in adults. In fact less than 30% of cases are described in the over 20ies. There are some reports in the elderly. When observed in adults the diagnosis is usually due to the onset of symptoms of associated pathologies or to complications such as cholangitis or cancer. Ultrasound, CT and ERCP allow, in the majority of cases, an adequate pre-operative characterization of the lesion. Intra-operative cholangiography verifies the completeness of surgery – always resective – and excludes residual biliary pathologies. The treatment in patients who already underwent conservative surgery is more complex, in fact, in symptomatic patients a second observation and resection is mandatory while in asymptomatic patients a careful and complete follow-up is adequate without underestimating the cancer risk.