Malignant rhabdoid tumor is a rare tumor occurring mostly in the neonatal kidneys and central nervous system. Cutaneous malignant rhabdoid tumors are extremely rare in adults. The aim of the study was to report on the clinical, histologic, and immunophenotypic characteristics of this cutaneous malignant rhabdoid tumor which developed in an adult. A 27-year-old male complained of a right palm neoplasm that had been present for 6 months, which was initially diagnosed as an epithelioid sarcoma by biopsy. However, detailed investigation with immunohistochemistry enabled us to make a diagnosis of a rhabdoid tumor. The patient underwent radical abrasion, chemotherapy, and irradiation, and has survived for 1 year without relapse. Only 20 adult cases have been reported thus far in the English literature. We are reporting the 21(st) case, who remains disease-free at 12 months. Complete resection and local irradiation may increase survival, because there is no standard and reliable curative chemotherapeutic regimen.