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Perilymphatic fistula (PLF) is an uncommon disease characterized by abnormal leakage of the perilymph from the inner ear into the middle ear. The symptoms of PLF include sudden-onset hearing loss, tinnitus, ear fullness, and vertigo resulting from inner-ear trauma, stapedial surgery or barotrauma such as valsalva, and nose blowing. As nystagmus can be induced by the affected-ear in down position, benign positional paroxysmal vertigo (BPPV) should be considered for differential diagnosis. About 40-50% of PLF patients have experienced spontaneous healing, but surgical repair should be considered when the hearing symptom and dizziness are not alleviated or are aggravated within a few days. Reported herein, with a review of the relevant literature, is a case of PLF initially misdiagnosed as BPPV but where successful fistula repair was finally achieved. Korean J Otorhinolaryngol-Head Neck Surg 2016;59(4):327-31