The angiographic and clinical findings in 17 young adults (14-39 years) with stenosis and/or occlusion of at least one of the common or internal carotid arteries were reviewed to determine the spectrum of carotid artery disease in this age group. Five aetiological groups were identified: atherosclerosis (3 patients), arteritis (4), non-inflammatory arteriopathies (6), compression by neoplasm (1), and occlusions of undetermined cause (3). Over a mean follow-up time of 19.7 months, 2 patients died (12.5%) and further cerebrovascular events occurred in 4 others (25%). We conclude that carotid angiography plays a meaningful role in providing a definitive diagnosis in young adults with carotid artery disease and thus may assist with therapy and prognosis.