Introduction
Neurofibromatosis Type 2 (NF2) is an autosomal-dominant tumor-prone disorder characterized by the development of distinctive nervous system tumors, including meningiomas, ependymomas and peripheral, spinal and cranial nerve schwannomas, in addition to skin anomalies and visual symptoms. Bilateral vestibular schwannomas are pathognomonic. So, the most common entry in the disease is through hearing impairment, which usually occurs in the second decade of life. But, unlike adults, children most frequently present with ocular, dermatological, and neurological symptoms. Brainstem ischemic syndrome, which is an under-recognized entity of unknown origin, occurs in teenagers without any previously known NF2 diagnosis, presenting as an acute or subacute event, which involves the midbrain or pons.