Intramuscular intestinal lipoma is one of the rare benign tumours of the small intestine in children and acts as a lead point of intussusception.We describe a case in which a 4-year-old boy presented with recurrent attacks of colicky abdominal pain and non-bilious vomiting. Abdominal examination was irrelevant. Per rectum (PR) examination indicated an empty rectum without red currant jelly stool and non-palpable mass. Abdominal ultrasonography revealed a target sign of intussusception. After a period of conservative treatment, the child passed normal coloured and well-formed stool. Exploratory laparotomy referred to a long segment ileo-ileal intussusception with a polypoid lipoma as a lead point. Microscopic examination showed a benign non-capsulated intramuscular ileal lipoma.Small bowel lipoma produces symptoms of intermittent bowel obstruction. Herein, the child presented with non- bilious vomiting, we attribute this to reflex sympathetic stimulation of the pylorus leading to pylorospasm.Small intestinal lipoma is a rare finding in children that may cause intussusception which does not resolve spontaneously.