Sclerosing hemangioma of the lung is a rare benign tumor that can metastasize to regional lymph nodes. It is a neoplasm that originates from type II pneumocytes and primitive respiratory epithelium. It is most common in middleaged women in form of peripherally localized node. Tumor is composed of two types of cells, and there are papillary, solid, sclerotic, and hemorrhagic histological pattern. Preoperative and intraoperative diagnosis of the tumor is difficult. In a 53-old female patient, radiologically was discovered hyperdense nodule with smooth margins, which was followed for two years, and then underwent enucleation of the tumor. The tumor was 25 mm in diameter and consisting of round and cuboidal cells. Microscopically, it showed papillary and solid arrangement. Based on immunohistochemical analysis, we made a diagnosis of sclerosing hemangioma of the lung. Three months after surgery, the patient was in good health condition. Because of the numerous differential diagnostic dilemmas, diagnosis is almost always based on permanent paraffin sections, using a wide range of immunohistochemical analysis. Type of surgery depends on the tumor location, and in case of larger tumors, lymph node dissection needs to be done as well.