Aneurysmal bone cysts (ABC) are benign tumors of the bones, formed by multiple cysts filled with blood and eroding the bone. They are rarely located at the skull. We report a case of a right temporal ABC in a 5-year-old girl. On admission, the physical examination showed a tough and fixed tumor, with no inflammatory signs nor vascular characteristics. The neurological examination was normal. Imaging studies showed an heterogeneous extracerebral mass, eroding the temporal bone and the greater wing of the sphenoid bone. The treatment consisted in an extensive tumor resection, associated with reconstructive surgery. The outcome was uneventful (follow-up 27 months). We underline the pathogenetic, diagnostic and therapeutic features of this condition.