Abstract
Von Hippel-Lindau (VHL) syndrome is a rare autosomal dominant disease with incomplete penetrance and variable expression. The features of cerebellar and spinal tumors, pheochromocytomas, and increased intracranial pressure complicate the anesthetic management of such patients. This report describes the anesthetic management of a parturient with VHL disease and highlights the importance of proper surveillance, vigilant management, and individualized treatment plans from a multidisciplinary team.