We report a 19-year-old woman who presented with headache, vomiting, and elevated blood pressure; pheochromocytoma and von Recklinghausen's neurofibromatosis were diagnosed. Her mother had the same skin lesions and was also found to have pheochromocytoma. Both patients underwent surgical resection and the postoperative courses were uneventful; the daughter subsequently married and delivered a healthy child. Although both pheochromocytoma and von Recklinghausen's disease are derived from neuroectoderm and are inherited disorders, concomitant familial occurrence of these two diseases is very rare, only three families have been reported previously worldwide.