Objective To improve the knowledge of pulmonary sclerosing hemangioma ( PSH)especially with bilateral multiple lesions of the lung. Methods The clinical data of 3 cases of PSH ( 1 case with bilateral multiple lesions in the lung) were analyzed, and the related literatures were reviewed. Results All of the 3 cases were females. A 22-year-old female patientwith bilateral multiple nodules in the lungs was complicated with thyroid multiple nodular goiter ( with hypothyroidism) , dysfunctional uterine bleeding ( with anemia) , nodular hyperplasia of the breast, and arteriovenous malformation over forearm. Thoracoscopicbiopsy of left lung and resection of the right pulmonary mass were performed and both the lesions were confirmed as PSH. The clinical manifestations of multiorgan diseases and the presence of PSH suggested Cowden syndrome in this patient. The other 2 cases aged 50 and 53 were asymptomatic with solitary pulmonary nodules identified incidentally. The accessory examinations for malignancies, infections, and autoimmune diseases showed no specific findings. Resection of the lesions were performed by thoracoscopic surgery and thoracotomy respectively, and the histopathological results proved to be PSH. Literature review showed that PSH typically occurred in middle-aged women without clinical symptoms and signs, often presenting as a pulmonary solitary nodule/mass identified incidentally. The differential diagnosis should include peripheral carcinoma, hamartoma, inflammatory pseudotumor and tuberculoma. Multiple PSH, which mainly presented as multiple well-defined nodules /masses of different size in the lungs, was rather rare, but easily confused with metastatic neoplasm. Lung biopsy by surgical operation was a common way to confirm the diagnosis, while FDP-PET and fine needle aspiration biopsy showed some defects. Surgical resection was an effective method of treatment, the residual lesions of multiple PSH should be monitored. Cowden syndrome may be considered if a PSH coexisting with abnormity of multiple organs such as thyoid, breast and vessels. Conclusions PSH should be considered during the differential diagnosis for solitary or multiple nodules /masses in the lung. Surgical biopsy is a common way to confirm the diagnosis. Local excision andnecessary follow-up are effective methods of treatment.