Spontaneous cervical artery dissection is increasingly recognized as a common cause of ischemic stroke in the young and middle-aged. Noninvasive imaging techniques such as magnetic resonance imaging and magnetic resonance angiography have widely replaced conventional angiography as the initial diagnostic study of extracranial dissections, allowing greater numbers of patients to be screened and thus leading to increased frequency of the diagnosis. We present a case of spontaneous carotid artery dissection in a previously healthy 48-year-old woman who presented with neck pain and elevated inflammatory markers. Marked gadolinium enhancement of the right extracranial internal carotid artery on magnetic resonance imaging led to an initial diagnosis of vasculitis. This case shows that the vessel injury associated with spontaneous carotid artery dissection is associated with an inflammatory response that can mimic vasculitis on highly sensitive imaging techniques, a phenomenon not well described previously. In this report, we review the nonvasculitic conditions that can mimic vasculitis and present clinicians complex diagnostic challenges. Recognition of these pseudovasculitic syndromes is important to avoid overdiagnosis resulting in unnecessary and potentially harmful immunosuppressive and cytotoxic treatments.