Mesenteric neurofibroma associated with von Recklinghausen's disease is rare. Herein, we present one such case. A 15-year-old mentally retarded Japanese boy presented with destruction of the right 2nd and 3rd ribs on a routine chest roentgenogram. Physical examination revealed a funnel chest and multiple cafe-au-lait spots, but no cutaneous nodules. Although the patient had no symptoms, a computed tomography (CT) and angiogram were performed. There were no definitive findings of malignancy in the tumors. However, since there were two risk factors for malignancy, specifically, a young age at the time of diagnosis and multiple tumors, and coupled with the size of the abdominal tumor which was large, the abdominal mesenteric tumor was removed. Pathological examination showed a neurofibroma with no evidence of malignancy.