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Peutz-Jeghers syndrome (PJS) is a rare autosomal dominant inherited disorder, manifested as multiple hamar-tomatous polyps of gastrointestinal tract, mucocutaneous pigmentations and increased risk of cancers. In this manuscript, we reported five cases of smal intestinal carcinoma associated with the PJS. Al the five patients have a history of PJS and postoperative pathological examination confirmed the diagnosis of smal intestinal carcinoma. Histopathological features and recommended surveilance were additionaly discussed.