Cystic vestibular schwannomas are a subtype of schwannomas characterized by a very aggressive and erratic natural history. In this chapter, we describe the pathophysiological, clinical, and diagnostic characteristics of vestibular schwannomas with cysts. The treatment is based on microneurosurgical excision through a retrosigmoid approach with transmeatal access. Although they were traditionally associated with a poor postoperative prognosis, it is now accepted that the results of microneurosurgical treatment do not differ from those of solid vestibular schwannomas of similar size.