Narrow internal auditory canal (IAC) syndrome is a malformation of the temporal bone, that is defined as an IAC diameter of only 1-2 mm on high-resolution computed tomographic scans (HRCT). This syndrome is known to be caused by the absence (aplasia or hypoplasia) of the vestibulocochlear nerve. We present a case of unilateral narrow IAC syndrome which was diagnosed by HRCT. The aplasia of the vestibulocochlear nerve was confirmed using parasigittal reconstruction magnetic resonance image (MRI). The IAC was composed of two separate canals, one of which contained a facial nerve and the other was empty with aplasia of the vestibulocochlear nerve.