We report the identification of a kaposiform hemangioendothelioma (KH) in the oropharynx of a 3-year-old boy. This is a rare endothelial-derived spindle cell neoplasm affecting children and early adolescents with features common to capillary hemangioma and Kaposi sarcoma. Nine cases of head and neck KH have been reported, this being the first in the otolaryngology literature. Our patient underwent wide local excision and has remained tumor free for over 1 year. KH should be considered in the differential diagnosis of a vascular lesion demonstrating unexpected behavior from that of a hemangioma.