Background: Lymphoepithelial cysts of the pancreas constitute a rare clinicopathologic entity. Case Report: We report a case of lymphoepithelial cyst of the pancreas and review the world literature. Results: Lymphoepithelial cysts are true pancreatic cysts lined by squamous epithelium and surrounded by mature lymphoid tissue. The cyst arises typically in middle aged men, and is usually asymptomatic or causes nonspecific abdominal complaints. There is no specific serologic marker for this entity. None of its radiologic characteristics can help differentiate it from other cystic lesions of the pancreas. Fine-needle aspiration cytology may be able to suggest its benign nature and identify it as a true cyst of the pancreas. The outcome after surgical excision is uniformly good with good symptom control and no recurrences. Recommendations: In the symptomatic patient or the asymptomatic patient with acceptable surgical risk a simple cyst excision should be performed after verification of the diagnosis with frozen section. In the asymptomatic patient with a high surgical risk, in whom fine-needle aspiration suggests the diagnosis of a lymphoepithelial cyst, observation of the lesion is recommended. When simple cyst excision is technically not possible, extensive resections/reconstructions should be avoided and drainage/bypass procedures may be considered.

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