AIM: Primary small bowel melanoma is a very rare clinical entity with a paucity of publications in literature. Most cases of gastrointestinal melanomas are metastatic lesions arising generally from primary lesion of the skin, eyes, or anus. We present a case of a small bowel intussusception with primary malignant melanoma as lead point and a gluteal melanoma metastasis after four years free from disease. CASE REPORT: A 77-year-old female has come to our attention with signs and symptoms of intestinal occlusion. She was subjected to a computerized tomography (CT) of the abdomen and pelvis that revealed small bowel intussusception caused by intestinal polypoid lesion. She was treated with a bowel resection. The histological exam has shown the presence of an amelanocytic malignant melanoma. The examination of skin, eyes, esophagus, colon and anus, a tot al body contrast-enhanced CT and a bone scintigraphy were negative for primary melanoma. So, the final diagnosis was primary melanoma of the ileum. After four-years disease-free survival, the patient came back to our attention for a gluteal melanoma metastasis, that was surgically removed. Afterwards she started immunotherapy, that is still ongoing. DISCUSSION AND CONCLUSION: The diagnosis and the treatment of primary intestinal melanoma is a challenging due to the lack of scientific indications. Our case shows how an early diagnosis, although accidental, can offer a good survival free from disease. Moreover, a careful follow-up of our patients allows us to promptly identify neoplasm recurrence or distant metastasis that can be treated with surgery and systematic therapy.