Rhabdomyosarcoma is the most common soft tissue sarcoma found in children. A polypoid form of the embryonal type of rhabdomyosarcoma, namely, sarcoma botryoides, is often found in girls younger than 5 years, and typically presents as a polypoid mass protruding from the vagina. Over the years, there has been a shift in the treatment of this condition from radical surgery to a multimodal approach involving conservative surgery with chemotherapy and radiotherapy. This approach has been associated with improved survival and preservation of normal anatomy and function. We discuss the initial management of a 4-year-old girl with pelvic embryonal rhabdomyosarcoma using a multimodal approach.