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Objectives: We report a case of primary melanoma of the small intestine. Primary intestinal melanoma (PIM) is an extremely rare neoplasm for which the cause is unknown.  Materials and methods: A 67-year-old man was admitted to our department due to abdominal pain, constipation, a large, hard inguinal mass and severe anaemia.  Results: After laboratory data, imaging techniques and histopathological examination, the diagnosis was confirmed. A surgical resection of the intestinal neoplasm, treatment with BRAF inhibitors and radiation therapy to the inguinal mass were performed. Conclusion: PIM is rare and it is usually difficult to establish its exact origin.