The occurrence of benign nerve sheath tumors within the neuroaxis is uncommon. Even rarer is the finding within brain parenchyma, termed malignant intracerebral nerve sheath tumors (MINST). We present a case of MINST which occurred in the frontal lobe of an 18-year-old male that recurred almost 4 years later. Imaging demonstrated a 4.0 cm lesion with an associated mass effect. He underwent a right fronto-parietal craniotomy for gross total resection. Pathology was inconclusive with a Glioblastoma Multiforme (GBM) as the most likely diagnosis, though gliosarcoma and MINST were also highly considered. Postoperatively, he was treated with chemotherapy and radiation and followed for almost 4 years, when an MRI indicated a recurrence. Resection of the recurrence was highly suggestive of MINST. Surgery was followed by radiation and chemotherapy, but, less than 7 months later, he was readmitted for a surgical-site infection, and, after multiple surgeries, and his family terminated care. Recognizing this unusual tumor in the differential diagnosis of a heterogeneously enhancing intracerebral mass can help surgeons diagnose and treat it. This report also exhaustively reviews the literature and presents diagnostic and treatment strategies.