Congenital segmental dilatation of the intestine is a rare disease. It is rarely located in the jejunum and its etiology is still unknown despite many theories suggesting its mechanism. We report a case of a 17 months girl who experienced nonspecific symptoms (abdominal pain, constipation and loss of appetite) since early her infancy. She had no growth retardation and had moderate abdominal distension on physical examination. Investigations undertaken could not increase suspicion of congenital segmental dilatation of the intestine (CSDI). The diagnosis was made peroperatively and a resection was done, followed by end-to-end jejunal anastomosis. There were no postoperative complications and the patient is doing well after four months. One should think of CSDI in children with chronic subocclusion or digestive hemorrhage.