BackgroundThe present study analyzes diagnostic and therapeutic surgical aspects of primary small bowel melanoma, describing the clinical case and reviewing the literature. Malignant melanomas represent 1–3% of all malignant tumors of the gastrointestinal tract and are therefore uncommon. Only a few cases of metastatic melanoma (1–5%) are diagnosed in the early stages, while still asymptomatic. They show up as imaging exam findings and have better chance of treatment. Most of the time, the diagnosis is late and made in the presence of complications. The final diagnosis frequently depends on the surgical intervention after a serious complication.Case reportThis case report refers to a 55-year-old male patient, complaining of abdominal pain, blackened stools, and palpable tumor in the abdomen for ~30 days. A tomography scan was performed, which revealed the thickening and parietal enhancement of the small intestine loops with mesenteric adenomegaly and intermingled collection. For diagnostic confirmation, a laparoscopy was performed, which revealed a tumor at the jejunal level, and its resection was performed in the same act. The anatomopathological examination revealed that it was a primary small bowel melanoma.ConclusionThe bibliographic research of the small bowel melanoma demonstrated that, in this situation, the lesion can be interpreted as a primary site or metastatic lesion, considering the possibility of a single primary lesion, whose diagnosis becomes more laborious. In such cases, adjuvant therapy must be considered. The expected 5-year survival is about 9–13%.