A 16-year-old girl applied to our ENT clinic with a 3-day history of right hearing loss, tinnitus, and pressure in the right ear. She had had surgery for right perilymph fistula two times, one at the age of 7 and the second at the age of 9. She had recovered after both of these surgeries. This time she had exploratory tympanotomy and perylymh fistula was detected. Computerized tomography investigation obtained after 5 days postoperatively showed bilateral large vestibular aqueducts and otherwise normal inner ear structures. Thyroid function tests and neck palpation were normal. It was an unusual case with both large vestibular aqueduct syndrome (LVAS) and simultaneous spontaneous perilymph fistula.