Embryonal rhabdomyosarcoma (ERMS) has a low prevalence, poor prognosis, and limited treatment efficacy. We report a case of an 18-year-old male whose disease relapsed in the abdominal cavity after a testicular ERMS curative resection. The patient received eight sequential cycles of rescue therapy using cisplatin and isocyclophosphamide in combination with a vascular targeted drug, Endostar. The therapeutic effect of the combination regimen has been evaluated for complete response. This is the first case to report using Endostar and chemotherapy in relapsed ERMS, and the curative effect results in complete response. Endostar, a new vascular targeted drug, combined with chemotherapy may play a synergistic role and provide a reference for the treatment of ERMS.