Mirizzi syndrome (MS) is a rare condition whose clinical presentation is unspecific, with obstructive jaundice being the most common form. MS is often not recognized at initial presentation, which can result in morbidity and biliary injury. Preoperative diagnosis is uncommon and over 50% of patients with MS are diagnosed during surgery. There are no clinical features to distinguish MS from cholangiocarcinoma, except that patients with cholangiocarcinoma are on average ten years older than patients with MS. We report the case of a 51-year-old female patient who presented with jaundice and weight loss. Following investigation with laboratory and imaging exams, the initial diagnosis was Bismuth II cholangiocarcinoma. The patient underwent surgical intervention, and anatomopathological examination of the specimen showed the correct diagnosis to be MS. Despite the rarity of its incidence, physicians must keep MS in mind as a possible differential diagnosis for cholangiocarcinoma and vice-versa. We discuss this case in the context of a brief review of the literature on Mirizzi syndrome mimicking cholangiocarcinoma.
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