| Summary: | Introduction. Choledochal cysts are congenital anomalies manifested as focal or diffuse cystic dilatation of the bile ducts. They are mostly diagnosed in childhood. The magnetic resonance and surgical management are the gold standard diagnostic and treatment modality. Case outline. We present a male patient who was presumed to have Mirizzi syndrome. This presumption was discarded by additional imaging procedures and by later surgical treatment. It was confirmed that it was a large choledochal cyst filled with stones. Considering the diagnosis and good patient’s general condition, we opted for surgical treatment. Conclusion. There are several surgical techniques that can be used in the treatment of choledochal cysts, wherein each is intended as a complete resection of the cyst with histological confirmation. Operative techniques do not affect the outcome of the treatment, but the time and extent of surgical resection, as well as any metaplasia of the epithelium, do.
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