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This cross-sectional study was designed to determine the prevalence of carotid artery atherosclerosis in patients with pulsatile tinnitus. All patients who had pulsatile tinnitus and presented to the otolaryngology clinic of Rasoul Akram Hospital, Iran University of Medical Sciences, and to some other private hospitals and clinics were referred to a tertiary referral center. From November 1999 to September 2003, 34 patients with pulsatile tinnitus underwent a clinical evaluation. Color Doppler ultrasonography was performed in all patients. Atherosclerotic carotid artery disease (ACAD) was found to be a cause of pulsatile tinnitus in four patients (11.76%). The mean age of these patients was higher than that of the other patients (59 vs. 34 years). All patients had at least one risk factor for ACAD. In three patients, pulsatile tinnitus was the first manifestation of ACAD; in two of these, this symptom occurred when the stenosis affected more than 70% of the artery's diameter. ACAD should be considered in evaluation of all patients with pulsatile tinnitus and associated cardiovascular risk factors. As pulsatile tinnitus may be the sole manifestation of severe carotid artery stenosis, immediate color Doppler ultrasonographic study is recommended in those patients with risk factors and in the elderly.