Our case joins several others with similar presentations and findings, including pediatric patients for whom brainstem ischemia was the presenting symptom of NF2.  The primary theory for the cause of brainstem ischemia in juvenile NF2 is a microvascular cause due to genetic deficiency of neurofibromin 2, a regulator of endothelial development, whose absence is likely to disrupt vascular homeostatic mechanisms.  Multicenter studies with large NF2 cohorts are needed to better characterize this syndrome.  Understanding brainstem ischemia in young NF2 patients will inform the development of treatment guidelines for such patients.