From the Department of Dermatology, College of Medicine, The Catholic University of Korea, Kangnam St. Mary's Hospital, 505, Banpo-Dong, Seocho-Gu, 137-040, Seoul, Korea, Gastrointestinal stromal tumors are usually late manifestations of neurofibromatosis (von Recklinghausen"s disease) and most become clinically apparent in middle-aged patients as multiple benign tumors. To our review of the literature, solitary malignant stromal tumor of gastrointestinal tract is exceptionally rare in von Recklinghausen"s disease. We herein present a case of solitary jejunal stromal tumor in a 50-year-old woman with NF1, which histopathologically showed a malignant change and combined smooth muscle-neural type.