A perilymph fistula is an abnormal connection between the inner and middle ear that allows escape of perilymph fluid into the middle ear compartment. The clinical symptomatology that follows leakage of fluid is by and large indistinguishable from a number of other pathologies that affect inner ear function. Definite diagnostic proof of a perilymph fistula remains elusive, and methods of diagnosis remain controversial. Traumatic tears in the oval or round windows remain a major cause of perilymph fistula, yet an index of suspicion in traumatic brain injury frequently remains low. The diagnosis of perilymph fistula must always be considered in the appropriate clinical setting of head trauma, barotrauma, and in patients with unresolved and undiagnosed episodes of recurrent vertigo or hearing loss. Surgical treatment with patching of oval and round windows remains the mainstay of therapy for this condition.