Sclerosing hemangioma of the lung is a rare and mostly benign lung tumor, which affects especially Asian middle-age women (median age of 48 years). We report the case of a 27-year-old woman in which, a premarital routine chest X-ray investigation revealed a 2 cm well-defined opaque nodule in the lower left pulmonary lobe, confirmed by CT scan. Microscopically, the surgically enucleoresected nodule was represented by a heterogenic tumor (papillary, solid, sclerotic, hemorrhagic patterns), containing two cell populations: cuboidal surface epithelial cells lining the papillary structures and round stromal cells in solid areas, with distinct immunoprofile and low mitotic activity, consistent with sclerosing hemangioma. This case is particular because, being rare in Caucasian persons, intraoperative diagnosis on frozen sections is extremely difficult, and routine histopathological diagnosis needs immunohistochemical tests to set the correct diagnosis, hence the correct therapeutic attitude.