Adrenocorticotropic (ACTH)-producing neuroendocrine tumours (NETs) are rarely found in the small bowel, and primary mesenteric NETs have only been reported in a few cases globally. We report the case of a 68-year-old female with ectopic Cushing’s syndrome due to excessive ACTH secretion from small bowel primary lesions and mesenteric metastasis. Initially, only the mesenteric mass was detected on imaging and endoscopy/colonoscopy, and it was only with surgical exploration that the small bowel lesions were found. This highlights the importance of high clinical suspicion and robust investigation when locating NETs. Surgical resection of the affected small bowel and mesentery was the definitive treatment for this patient. Initial hydrocortisone replacement therapy was needed, and subsequent biochemical tests and clinical reviews demonstrated no recurrence.