The primary malignant tumors of the small bowel are rare, representing 1 to 1.4% of all gastrointestinal tumors. We report a case of a 33 year-old women, admitted to our emergency department of visceral surgery for acute abdomen. The clinical examination revealed diffuse abdominal distension, defenseless, the hernia orifices were free and the rectal examination was normal. The biological test showed no hydro electrolytic disorders with normal hemoglobin and normal renal function. The abdominal CT-Scan showed signs of bowel obstruction due to a volvulus with intussusception without ischemia. The patient was operated urgently; the exploration has revealed a small bowel obstruction in the ileum with volvulus, an intussusceptum associated with a retractile mesenteritis, and the hepatic exploration found no metastases. The patient underwent a bowel resection taking away the intussusceptum with the infiltrated mesentery. The postoperative course was uneventful. The pathological result has proved a well-differentiated neuroendocrine tumor with five free nodes. Through this observation, we aim to highlight that an obstruction of small bowel with volvulus and intussusception could be exceptionally due to a neuroendocrine tumor, this complication has enabled a relatively early diagnosis in the absence of metastases and a 6-month follow-up without recurrence is a demonstration.