Perilymphatic fistula (PLF) is an abnormal connection between the inner ear and middle ear space which allows the escape of perilymph and may result in disorders of hearing, balance, or both. This entity has received considerable attention and is a major area of interest and controversy in modern otology. The clinical manifestations of this condition are variable and encompass the whole spectrum of audiovestibular signs and symptoms. The diagnostic criteria have yet to be established. Currently there is no consistent objective preoperative test to diagnose PLF. Definitive diagnosis is made on middle ear exploration and rests on the observation of the surgeon. There is no objective intraoperative test to confirm the presence of PLF. The purpose of this paper is to present the classification system and current understanding of the pathogenesis of PLF. In addition, clinical manifestations, evaluation, and management of patients with this otologic entity will be reviewed.