Perilymph fistula, an abnormal communication between the inner ear and the middle ear, is an enigmatic otologic disorder which may present with auditory or vestibular symptoms. It is typically located at the oval or round windows, but several other locations have been suggested. It may be congenital or spontaneous, or due to trauma or surgery. It often poses a diagnostic dilemma because of the lack of a definitive test. Among the differential diagnoses of the perilymph fistula are Ménière's disease, infection, tumor and central nervous system disorders. Clinically, the definitive diagnosis of a perilymph fistula can only be made by exploratory tympanotomy. A current review of the etiology, diagnosis and management of perilymph fistula is presented.