Neoplasia and endocrine disorders are common complications of neurofibromatosis 1 (NF1 - von Recklinghausen disease). We report here a case of NF1 in a 31-year-old woman presenting simultaneously with an abdominal neurofibrosarcoma and a pheochromocytoma. The prevalence of secondary neoplasia and endocrine tumors in NF1 is also discussed.