Background. Hemangiondothelioma is a rare vascular tumor that can occur in the bone. Temporal bone involvement has been reported extremely rare in the literature.

Case. Radiological examination of a one-year-old girl who was admitted due to facial paralysis revealed vascular tumor of the temporal bone and Galen vein aneurysm. Pathological examination showed retiform hemangioendothelioma. She was treated with propranolol, prednisolone, vincristine, and endovascular embolization followed by oral sirolimus. With sirolimus treatment, a partial response was obtained first, then the tumor remained stable and sirolimus treatment was discontinued. No progression was observed in the disease after discontinuation of treatment.

Conclusion. In this article, a case of hemangioendothelioma originating from the temporal bone is discussed in the light of other case reports in the literature.

Keywords: childhood, galen vein aneurysm, hemangioendothelioma, sirolimus, temporal bone