The aim of the present study was to summarize the clinical feature of the perilymphatic fistula. Charts of 249 patients with various ear symptoms followed by exploratory tympanotomy were retrospectively analyzed. In 23% of patients a visible fistula in the round or/and oval windows was identified. The majority of patients reported a history of pressure elevation and minor head injury. Vertigo improved in 82% of the patients and the hearing loss in 11% of the patients. Methods of diagnosis of perilymphatic fistula still remain controversial. This manuscript also reviews the various diagnostic methods such as the highresolution computed tomography, magnetic resonance imaging (MRI), and CTP.