We reported one case of a primary liposarcoma of the lung which has been reported only in six cases to date worldwide, and we added some documented study. A 49-year-old female complained of exertional dyspnea with about 100 ml of hemoptysis. The chest X ray showed a coin lesion at the left upper field. After left pneumonectomy, the histological examination revealed liposarcoma of the lung. Six months after the operation, she died from severe dyspnea; the autopsy revealed the relapse of liposarcoma in the right lung, and no liposarcomas in other organs. This is the seventh case according to a worldwide review of the literature.