Abstract： Objective To analyze the clinical features of one case of spontaneous hemothorax caused by primary pleural chondrosarcoma and therefore to improve the understanding of this disease.Methods The clinical features of a case with primary pleural chondrosarcoma were analyzed retrospectively and the related literatures were reviewed.The literature review was carried out with "primary pleural,chondrosarcoma" in Chinese and English respectively,as the search terms in Wanfang Data,CNKI and PubMed database from January 1980 to October 2015.A total of 6 articales,1 in Chinese and 5 in English,were reviewed.Results A 29 year-old male patient was admitted to the hospital because of fever,chest tightness,shortness of breath for 20 days.CT scan of the chest showed a mass near the right posterior fourth rib and right pleural effusion.Routine examination of the pleural effusion confirmed the presence of hemothorax.Thoracotomy was performed and revealed hemothorax in the right thorax,and a mass near the pleural apex.The tumor was removed by surgery and pleural decortication was also performed.Pathology study confirmed the diagnosis of high-differentiated chondrosarcoma.The patient was followed and there was no recurrence until now.A total of 6 case reports were retrieved from Wanfang Data,CNKI and PubMed.Five cases had complete data,including 2 males and 3 females(age from 28 to70),and another (a 78-year old male) without adequate data.Conclusions Primary pleural chondrosarcoma is a rare disease,and hemothorax as the first manifestation is even rare.It is easily to be misdiagnosed due to nonspecific clinical symptoms.The final diagnosis depends ultimately on pathological biopsy.Thoracotomy is the most effective method for treatment of primary pleural chondrosarcoma.