Malignant rhabdoid tumor (MRT) was first described as a variant of Wilms' tumor but was subsequently found to be a highly malignant tumor composed of primitive cells that resemble rhabdomyoblasts. In the past decade, extrarenal MRTs were reported in different locations and organs throughout the body including the female genital tract. We here report an MRT that arose in the corpus uteri, the second such case reported.