A 41-year-old female with 'cafe au lait' and multiple skin neurofibromas was admitted with a complaint of constipation, abdominal pain and distention. The colonoscopic investigation was normal, however computed tomography revealed a mass in the cecal wall;Thereon, right hemicolectomy was performed to the patient. Histopathologic examination revealed ganglioneuromatosis of the cecum. There was not any recurrence after three years of follow-up. An intestinal manifestation of von Recklinghausen's disease should be kept in mind in a patient who has multiple neurofibromas and 'cafe au lait' spots with intractable constipation and intestinal obstruction.