Based on reported a case of pleura rhabdomyosarcoma and reviewed the above mentioned diseases in recent years, to summarize the clinical features, diagnosis and treatment of this disease for improve the understanding of this disease.

Reported a case of pleura rhabdomyosarcoma that we diagnosis and treatment, and retrieval of similar cases in 20 years in literature. Then summarizes the clinical features, diagnosis and treatment. Case: 65 year old male patient with right chest pain in 1 month, with irritating cough and a small amount of white mucus sputum, chest radiographs: the lung texture is increase, right pleural has a visible large high density shadow, right upper lung lobe shows flocculent slightly higher fuzzy density. PET-CT shows right pleura lesion has a nodular soft tissue density shadow, which has increased radioactivity. And some lymph nodes in Mediastinal are larger than normal. Color ultrasound examination of pleural effusion shows right pleura lesion detects a huge substantial tumor.

The centesis biopsy was confirmed as rhabdomyosarcoma. Patient was not treated and leaves hospital after diagnosis. After a few days the disease progressed and the patient died at home, the direct cause of death is unknown.

The incidence rate of pleura rhabdomyosarcoma is very low; The clinical and imaging manifestations of pleura rhabdomyosarcoma are not specific. Chest radiographs are mainly performance as lung high density shadow. The diagnosis mainly depends on pathology, and is easy to be misdiagnosed as other common lung cancer. It should be paid enough attention to these diseases in clinical practice.