Neurofibromatosis type 1 (NF1) is a multisystemic disease with an autosomal dominant pattern of inheritance, complete penetrance and variable expressivity. This disease affects one in every 3,000 individuals and one out of every 200 patients with mental retardation. Gastrointestinal bleeding secondary to gastrointestinal mesenchymal tumors is one of the many complications associated with NF1. We report the case of a patient with NF1 admitted to hospital for severe gastrointestinal hemorrhage secondary to a jejunal gastrointestinal stromal tumor (GIST) with favorable outcome after surgical removal.