A case of acute gastrointestinal haemorrhage in a patient with systemic neurofibromatosis is presented. The bleeding was localized using mesenteric angiography and small bowel enema to a segment of ileum with multiple smooth muscle neurofibromata. The patient had a complete recovery after the resection and remained asymptomatic at 2 years follow up. Small bowel neurofibromatosis is a rare but important differential in gastrointestinal haemorrhage. Early diagnosis and surgery are vital for patient survival.