Neurofibromatosis type 1 is an autosomal dominant disease affecting one in 3000 to one in 4000 people, with a great variability of clinical expression. Individuals affected with neurofibromatosis type 1 have an increased risk of developing both benign and malignant tumors, supporting the classification of tumor predisposition syndrome. The most common tumor is the neurofibroma, a heterogeneous benign nerve sheath tumor, which represents the primary clinical characteristic of neurofibromatosis. The case reported refers to a adolescent boy with neurofibromatosis type 1 diagnosed at 20 months, who presented progressive growth of dorsal and lumbar intraspinal tumors since six years of age and diagnosis of malignant nerve sheath tumors at 17 years of age. In addition to describing a rare presentation of neurofibromatosis, because of location and early onset of complications, the authors discuss the difficulties of the therapeutic approach of this case.