We present the case of a 3-year-old female who presented with a vaginal mass and was subsequently diagnosed with intermediate risk, stage I, group III vaginal embryonal rhabdomyosarcoma. She was staged and treated with chemotherapy according to the Children’s Oncology Group protocol. Surgical management included initial tumor debulking and subsequent partial vaginectomy with vaginal reconstruction following chemotherapy. There has been no evidence of disease on surveillance 7 years after diagnosis.