Rhabdomyosarcoma (RMS) is a common soft tissue sarcoma usually observed in children. However, RMS rarely occurs in adults. The prognosis of adult RMS is poor and a standard chemotherapy regimen has not yet been established. Herein, we report the case of a 60-year-old Japanese woman with primary anterior mediastinal alveolar RMS (T3N0M0, stage III). The tumor increased aggressively despite first-line treatment with doxorubicin (60 mg/m 2 every 3 weeks for 1 cycle) and second-line treatment with eribulin (1.4 mg/m 2 every 3 weeks for 2 cycles). Although her shortness of breath and chest tightness worsened as the tumor compressed her heart and left main bronchus, and her performance status (PS) decreased to 3, third-line treatment with pazopanib (800 mg once daily) was commenced. The treatment led to suppression of tumor growth and resulted in 4-month progression-free survival. Therefore, in cases of adult RMS, considering pazopanib treatment as an option may be beneficial, even with previous ineffective treatments or poor PS.