The case of a 17-year-old patient with progressive unilateral sensorineural hearing loss and temporal bone malformations concerning for internal auditory canal meningocele with translabyrinthine/perilabyrinthine cerebrospinal fluid fistula is presented with associated computed tomography and magnetic resonance imaging. As the second reported case of an unruptured internal auditory canal meningocele with translabyrinthine/perilabyrinthine fistula, the case presents several clinically relevant points for otologists, neurotologists, and neuroradiologists. Although rare, it is an additional entity to consider as a cause of unilateral sensorineural hearing loss and may pose a risk for developing meningitis and possible "gushing" of cerebrospinal fluid should surgical intervention be attempted. Laryngoscope, 126:1931-1934, 2016.