The posterior fossa is an intracranial compartment that houses the cerebellum and the brainstem. Mass lesions and increased intracranial pressure (ICP) in this area can have profound consequences for neurologic, cardiac, and respiratory functions. This chapter presents a case study of a 54-year-old female with complaints of headaches, progressive hearing loss and episodes of aspiration that were increasing in frequency. A diagnosis of neurofibromatosis type 2 was made and a posterior fossa craniotomy was scheduled. Immediate concerns for the anesthetic team included (1) a depressed level of consciousness, (2) the potential for increased ICP, and (3) aspiration on induction. Neurosurgical procedures involving the posterior fossa can be challenging for both the surgical and anesthetic teams. Preoperative assessment and preparation of these patients should focus on the consequences of posterior fossa abnormalities as well as the potentially life-threatening complications that may occur intraoperatively.