Abstract
Neurofibromatosis type 1 (NF1), also known as Von Recklinghausen disease, is a multisystemic, hereditary, autosomal dominant condition. It is caused by the development of tumors in the nervous system, resulting from mutations in the NF1 gene located on chromosome 17q11.2. Gastrointestinal involvement is mainly extraperitoneal, with mesentery lesions being the least common. We report a case of an isolated jejunal mesenteric neurofibroma diagnosed by ultrasound and CT scan and confirmed by histopathological examination in a nine-year-old child with diffuse café-au-lait spots who presented with chronic abdominal pain.