We describe the case of an 18-year-old female with Cowden syndrome in whom a simple x-ray detected a solitary pulmonary nodule that was identified as a sclerosing hemangioma. Pulmonary sclerosing hemangioma is an unusual lung neoplasm which typically presents as a solitary peripheral nodule in asymptomatic women. Although the histology of this entity is well defined, its origin and treatment is debated. One of the main diagnostic problems is to histologically differentiate a pulmonary sclerosing hemangioma from a papillary lung carcinoma.