Myxoid leiomyosarcoma is an extremely rare variant of leiomyosarcoma, masquerading almost to perfection as a benign lesion. For, indeed, the tumor lacks the defining features of high mitotic activity, cellular atypia or necrosis, and the microscopic picture is dominated by abundant myxoid stroma containing sparse spindle cells. We report here such a case occurring in the uterus and discuss the differential diagnosis. The relevant literature is reviewed.