Introduction: The aneurysm or loops of the anterior inferior cerebellar artery (AICA) in the internal auditory canal are exceeding rare and its clinical manifestations vary. The vascular loop of the AICA in the internal auditory canal may result in compression of the eighth or vestibulocochlear nerve. Microvascular compression of the eighth cranial nerve often causes tinnitus, hearing loss and vertigo. Several works of literature are reporting the microvascular compression of the vestibulocochlear nerve at the cerebellopontine angle (CPA) and not intrameatal area. Aim: To report an unusual cause of unilateral profounding hearing loss and disabling tinnitus. Case Report: A 42-year-old man was presenting with hearing loss and tinnitus in the left ear for 2 months. MRI confirmed the intrameatal compression of the vestibulocochlear nerve by the vascular loop of the AICA. Discussion: The neurologic examinations of the patient were within normal limits. The caloric test was normal on both sides. Auditory brainstem response (ABR) showed latency of wave I and III brainstem potentials.MRI showed the vascular loop of the AICA compressing the vestibulocochlear nerve. Conclusion: Vascular loop by AICA in the internal auditory canal can compress the cochlear nerve which results in cochlear symptoms like tinnitus and sensorineural hearing loss. This vascular malformation can be diagnosed with help of clinical symp toms and magnetic resonance imaging (MRI).