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BACKGROUND Primary intracranial rhabdomyosarcoma is a very rare malignant tumor. Owing to moderately vascular and firm characteristics of the tumor, complete removal without complications is often difficult. In pediatric patients, the volume of total blood is less than in adults, and minimal intraoperative hemorrhage is desirable. CASE DESCRIPTION A 6-year-old boy presented with ataxia and was found to have a large cerebellopontine angle tumor and hydrocephalus. The patient underwent preoperative coil embolization of the anterior inferior cerebellar artery to decrease tumor vascularity, and gross total removal was achieved on the same day via a suboccipital approach. Pathologic diagnosis was rhabdomyosarcoma. CONCLUSIONS Successfully resection of primary intracranial rhabdomyosarcoma was achieved after preoperative embolization. Tumor vascularity was significantly reduced, and gross total removal was achieved safely with less hemorrhage.