Advanced small cell carcinoma of the ovary is very aggressive tumor with an overall poor prognosis. The authors describe an 11-year-old girl who presented with advanced-stage disease. She was treated with gross total surgical resection and multiagent chemotherapy and remains free of disease more than 2.5 years from diagnosis. The treatment regimen is discussed in detail and was well tolerated, with minimal toxicity. This is the first case report of a pediatric patient with advanced-stage disease to achieve long-term survival.