Posterior fossa lesions can occur in patients of all ages. Patients present with subtle findings of hearing loss, tinnitus, headache, cranial neuropathies, imbalance, vertigo, nausea, and vomiting depending on the location and type of lesion. The presentation can be particularly insidious as the most common symptom is slowly progressive hearing loss. Posterior fossa lesions should be included in the differential diagnosis for patients with this presentation and appropriate audiologic testing and imaging should be performed. Management involves collaboration between multiple subspecialties and has evolved significantly over the years leading to increased patient involvement and improved outcomes.