Abstract: Objective：To investigate the efficacy and safety of VAC/IE regimen in the adult patients with advanced rhabdomyosarcoma. Methods：A total of 16 adult patients with advanced rhabdomyosarcoma and a median onset age of 31 years old, admitted to the Department of Medical Oncology, Zhongshan Hospital, Fudan University from January 2010 to December 2018 were enrolled. All patients were treated with VAC/IE regimen for the first-line palliative treatment. The efficacy and toxicity of the regimen were analyzed. Results：The average follow-up time was 13 months. The overall response rate (ORR) was 37.5% (6 cases) and disease control rate (DCR) was 68.8% (11 cases). The median progression-free survival (PFS) was 5.8 months, while the median overall survival (OS) was 15 months. All patients received a total of 93 cycles of chemotherapy. Among which, the adverse reactions grade 3 or above were mainly neutropenia (34/93, 36.6%), nausea and vomiting (27/93, 29.0%). Conclusions：The rhabdomyosarcoma is a highly malignant tumor and rare in adults with poor overall prognosis. For advanced patients, alternating of palliative chemotherapy with VAC and IE is effective and feasible based on increasing leukocyte and stopping vomiting.