We present a brief review of sclerosing hemangioma, an uncommon but histologically distinctive neoplasm of the lung. Based on immunohistochemical and molecular findings, sclerosing hemangioma is thought to be derived from incompletely differentiated respiratory epithelium. Sclerosing hemangiomas typically present as asymptomatic, peripheral, solitary, well-circumscribed lesions in women with a mean age at diagnosis in the fifth decade. Rare cases are reported to have regional lymph node metastases; however, metastases do not appear to affect long-term survival. Histologically, sclerosing hemangioma is characterized by a distinct constellation of findings including 2 epithelial cell types, surface cells and round cells, which form 4 architectural patterns, papillary, sclerotic, solid, and hemorrhagic. Sclerosing hemangioma of the lung is generally considered to be a benign lesion, and surgical excision is curative without the need for additional treatment.