Kaposiform hemangioendothelioma is a rare vascular tumor and locally aggressive endothelial-derived spindle cell neoplasm, which occurs almost exclusively in infants and adolescents. Radiologically, hemangioendothelioma, including Kaposiform hemangioendothelioma, is seen as a highly vascularized well-enhancing tumor, but no characteristic findings differentiate Kaposiform hemangioendothelioma from other soft-tissue tumors, particularly when the tumor is too small to have any locally aggressive features or identifiable large vessels. We present a case of Kaposiform hemangioendothelioma in the internal auditory canal that had no differential features on initial MR images and rapidly grew into a huge mass in a few months.