Kaposiform hemangioendothelioma (KHE) appears as a single lesion at birth or early infancy in an equal sex ratio. A rare aggressive vascular proliferation has been recognized as a separate entity from other childhood vascular neoplasm. A 3-year-old Iranian boy with a rapidly enlarging mass in his (posterior aspect of skull at the midline) skull was present here. Physical examination revealed a dark-red, firm mass measuring 5 × 4.5 cm in the posterior aspect of skull. No association with Kasabach- Merritt syndrome (KMS) was observed despite its size. Histologically, KHE was composed of infiltrating nodules with slitlike or crescentic vessels that are poorly canalized and lined by spindled endothelium cells. Immunohistochemically, both spindle and epithelioid cells were immunoreactive for CD34 and CD31, while negative for EMA, cytokeratin or S100 protein. α- SMA were detected in pericytes surrounding spindle cells. Recurrence occurred 2 month after first operation. Wide resection was performed at second operation and the patient was still alive during the 1-year follow- up period.