OBJECTIVE To report a rare case of pleomorphic rhabdomyosarcoma which occurred in the mediastinum of a 34-year-old man. CLINICAL PRESENTATION AND INTERVENTION A young male labourer presented with dyspnoea on exertion. A large mediastinal mass was detected on chest CT scan. The chest surgeons advised against open biopsy. His alpha-fetoprotein was 22,000 IU/l; based on this the diagnosis of a germ cell tumour was made and the patient was treated with a bleomycin/etoposide/cisplatin regimen. He left for his native country where an open biopsy from the mediastinum was taken and reported as pleomorphic rhabdomyosarcoma. He was given five courses of chemotherapy with doxorubicin, etoposide, and ifosfamide with mesna protection without much relief. The inoperable disease occupied the whole of the right chest and mediastinum. The enormous size of the radiation field made radiotherapy prohibitive. Finally, the patient opted for symptomatic treatment and left for his native place. CONCLUSION This case is presented because of its difficulty in management and rarity.