Perilymph fistula (PLF), an abnormal communication between the inner and middle ears associated with permanent or transient perilymph leakage, differs from cerebrospinal fluid fistula. PLF was first described after stapedectomy, and posttraumatic and spontaneous or idiopathic forms have since been reported. It is the source of various cochlear and vestibular symptoms. Diagnosis and surgical treatment remain controversial, especially for spontaneous PLF. Preoperative tests and imaging techniques have proved inadequate, and there is in fact no single diagnostic test for PLF. Therefore, the patient’s history and clinical picture still provide the main basis for diagnosis, which can be confirmed by careful exploratory tympanotomy (though this is not always foolproof). β2-Transferrin analysis allows objective confirmation in debatable cases. The indication for exploratory tympanotomy and surgical treatment depends on the etiology of PLF and the clinical picture. Treatment requires sealing of the fistula by connective tissue or stapedectomy. Vestibular symptoms show improvement in more than half of the cases, whereas auditory symptoms are generally less responsive to surgical repair. The diagnosis and rationale for surgical treatment are still based on clinical criteria. There is a need for multicenter studies to improve diagnostic testing and management of the disorder.