Primary pulmonary rhabdomyosarcoma (PPRMS) is a rare malignant tumor of mesenchymal origin, which is commonly found in minors and is usually found in the face, head, neck and limb regions. However, the incidence of primary rhabdomyosarcoma within the lungs of elderly patients is exceedingly uncommon. This case report describes an elderly male patient diagnosed with primary rhabdomyosarcoma in his lung, who was considered a lung cancer patient at primary diagnosis phase. Additionally, the report summarizes the differential diagnosis between primary pulmonary rhabdomyosarcoma and primary lung cancer.