Background: Amphicrine tumors of small bowel are rare lesions of the gastrointestinal tract that exhibit both neuroendocrine and exocrine features. It is an uncommon lesion with distinct biological and histological features. No clear guidelines on management of amphicrine carcinomas have been documented so far. Case Report: Here we report the case of a 60-year-old female who presented with abdominal pain and weight loss. Initial imaging suggested terminal ileitis favouring Crohn’s disease or tuberculosis. She underwent a limited ileo-caecal resection and the postoperative histopathological examination unexpectedly revealed neoplastic cells with both neuroendocrine and exocrine components, consistent with amphicrine carcinoma. Postoperatively, she is on regular follow-up without any recurrence. Conclusion: This report aims to help physicians understand the disease better, and to differentiate it from other mixed tumors in order to reduce the rate of clinical and pathological misdiagnosis.