Clear cell sarcoma also known as malignant melanoma of soft parts is a rare soft tissue neoplasm with melanocytic differentiation. It occurs predominantly in the soft tissue of extremities, typically involving tendons and aponeuroses of young adult. Primary clear cell sarcoma of visceral organ is extremely rare. We report here a case of primary clear cell sarcoma arising in the uterus of 65-year-old woman. To the best of our knowledge, this is the first report of clear cell sarcoma in the uterus. Aspects of clinical presentation, histopathologic evaluation and brief review of a literature are also described.