Hemangioendothelioma (HE) is a rare vascular tumor of endothelial cell origin that usually involves long bone or soft tissue. However, there are cases of head and neck involvement. In the literature, only 19 cases of nasal involvement are described. We report a case of an 18-month-old child who had HE of the ethmoid sinus. Endoscopic resection of the tumor was performed. The patient is well without any recurrence after 4 years of follow-up. Because of its rarity, the literature on HE in the nasal cavity and paranasal sinus is reviewed and its management is discussed.