Common and uncommon nonatheromatous diseases affecting the cervical carotid artery are discussed. Specifically, the radiographic and pertinent clinical features of cervical carotid artery congenital variants, dissection, fibromuscular dysplasia, Takayasu's arteritis, and infection are described. Catheter angiography continues to have an important role in the diagnosis of many of these conditions. Magnetic resonance imaging techniques are emerging as a useful adjunct to catheter angiography and occasionally may be the primary imaging modality for diagnosis.