Abstract

Neurofibromatosis type 1 (NF1), also called Von Recklinghausen disease, is the most common genodermatosis. Its incidence; prognosis and genetic mechanisms have been the subject of many publications. NF1 patients have an increased risk for developing benign and malignant tumors. Gastrointestinal stromal tumors (GIST) are rare type of cancers, potentially malignant. Patients with NF1 are much more likely to develop GIST with an incidence of 6% to 7% and a median age of 65 years. Cancers are the leading cause of premature death in patients with NF1. We report the case of a young man with neurofibromatosis type 1 who developed a metastatic GIST.