We reported a case of anterior inferior cerbellar artery infarction initially manifesting with positional vertigo ensuing from abrupt onset of hearing loss. A 56-year-old Taiwanese man suffering from controlled hypertension visited a local hospital because of a “rocking sensation.” He was referred to the ear, nose, and throat (ENT) department for evaluation and was diagnosed with peripheral vertigo associated with positional change. Symptomatic treatment was administered, but produced a limited effect. He was then transferred to our hospital and was admitted to ENT services. The symptom was aggravated when the patient turned his head to either side or bent his neck forward. Two days after admission, he suffered from abrupt onset of hearing loss in the left ear. A neurologist was consulted. Neurological and physical examinations revealed unremarkable findings, except for sensorineural type hearing impairment in the left ear. Cerebral magnetic resonance imaging (MRI) disclosed left-side anterior inferior cerbellar artery territory infarction. Magnetic resonance angiography (MRA) indicated stenosis in the midportion of the basilar artery and poor visualization of the left anterior inferior cerebellar artery. Auditory brain stem evoked potential testing indicated severe peripheral-type auditory pathway disorder in the left ear. Our patient was treated with 100 mg of lose dose Aspirin once daily for one month during hospitalization. Hearing loss was partially improved, whereas positional vertigo remained refractory. The patient was still undergoing regular follow-ups at neurological clinics up to date. This case indicated the urgency of neurological consultation when first-line clinicians encounter prolonged positional vertigo accompanied by sudden hearing loss. A high index of suspected ischemic anterior inferior cerebellar artery infarction should be considered and be placed into differential diagnosis. We recommend that neuroimaging studies be ordered in any case