Primary intracranial rhabdomyosarcoma of the cerebellopontine angle mimicking a vestibular schwannoma in a child

Primary intracranial rhabdomyosarcoma (PIRMS) is a rare neoplasm, which affects infants and young children. We report a rare case of a primary embryonal rhabdomyosarcoma of the cerebellopontine angle in a 7-year-old boy with clinical and radiological features mimicking a vestibular schwannoma. The patient underwent definitive surgery and radiotherapy and is recurrence free at 6 months. PIRMS commonly occur in parameningeal locations or metastasize to the brain from an extracranial primary. Gross total tumor resection followed by adjuvant radiotherapy remains the current standard of treatment; however, chemotherapy has also been tried with favorable results. The overall prognosis continues to remain poor.