Fifteen patients with bilateral acoustic neuromas are presented. Ten patients became symptomatic before age 21, and 9 of these patients developed additional central nervous system tumors. Cutaneous manifestations of neurofibromatosis and a positive family history of this disorder were absent in many of the patients. Most patients required multiple surgical procedures, and the timing, techniques, and results of these operations are reported. The pathogenesis of these tumors, the genetic aspects of this disorder, and potential treatment alternatives are discussed.