The arteriographic diagnosis of spontaneous, nontraumatic dissection of the internal carotid artery was made in 19 patients and confirmed at operation in ten. The tapered narrowing beginning in or about the carotid bulb and ending at the bony canal was a consistent finding. Four patients had associated aneurysm formation. All but one patient developed an acute hemispheric neurologic deficit as the initial symptom. The deficit was transient in ten and prolonged in eight. The dissection occurred in the outer layers of the media. None of the surgical specimens showed atherosclerosis. Surgical methods of management included segmental resection and grafting, thrombectomy and intimectomy, dilation, and simple ligation. Considerable improvement of luminal diameter occurred in six of seven patients whose arteries were left undisturbed.