Malignant transformation of a schwannoma (neurilemoma) is an exceedingly rare event. We describe two cases with such change and review the reported purported examples. The tumors in our patients involved a finger and pelvis. Sex, age, and clinical follow-up were available for only the second case, involving a 31-year-old man who died with recurrent and metastatic tumor. Seven acceptable cases were found in the literature. Analysis of the nine cases of schwannoma with malignant transformation showed no sex predilection, but revealed a tumor differing significantly from conventional malignant peripheral nerve sheath tumors. The mean age (56 years) was two decades older, no patient had neurofibromatosis, in four cases there was a years-long history of an antecedent mass, and in none of the cases was the malignant component an interlacing, fasciculated spindle-cell tumor. Rather, the malignant component was commonly purely epithelioid (seven of nine cases). In the two other cases, cells of the malignant component had neuroepithelial features. The prognosis for patients with schwannomas undergoing malignant change is poor. Five of eight patients with follow-up (62%) died of disease with either residual (one patient) or metastatic tumor (four patients).