To define the clinical characteristics of cystic acoustic neuroma, we retrospectively analyzed 22 patients with cystic acoustic neuroma and reviewed the literature with regard to clinical manifestation, imaging features, diagnosis, surgical procedures and prognosis. An acoustic neuroma was defined as cystic according to the following criteria: the presence of hypodense/hypointense areas on CT or MRI, the identification of cystic elements at operation and histological verification. At the end of surgery, the facial nerve was anatomically intact in 86.4% of cystic acoustic neuromas. Complete removal of the tumor was achieved in 18 cases (81.8%). We conclude that patients with cystic acoustic neuroma need prompt surgery with special attention paid to the preservation of the facial nerve.