RESUMEN
BACKGROUND: Primary central nervous system sarcomas are rare primitive mesenchymal non-meningothelial tumors. Malignant peripheral nerve sheath tumor accounts for 5% of sarcomas, with an incidence of approximately 0.001% and a recognized association with neurofibromatosis type 1. Its intracranial subtype, the so-called malignant intracerebral nerve sheath tumor (MINST), is even more infrequent. Current knowledge about its clinical presentation and best therapeutic management is poor because of the limited number of cases reported in literature. Commonly, intratumoral hemorrhage occurs at the time of diagnosis and, notably, most patients had intracranial hemorrhage prior to definitive diagnosis. CASE DESCRIPTION: We report a case of MINST in a young boy affected by neurofibromatosis type 1 who presented a spontaneous intracranial hemorrhage, successfully treated with surgery and postoperative adjuvant therapy. The tumor relapsed 1 year after and was successfully retreated with a second surgery. CONCLUSIONS: Malignant intracerebral nerve sheath tumors are rare sarcomas that can be associated with intratumoral hemorrhage at the time of presentation, mostly in patients with neurofibromatosis type 1. Surgery promptly performed, associated with adjuvant therapy, can result in an encouraging survival rate.