Headache is a common complaint among patients seeking medical assistance. The differentiation between a primary headache disorder versus headache as a symptom of a serious underlying disease is of crucial importance. Dissections of the carotid or vertebral arteries frequently present with headache and can result in ischemic stroke. Rarely, headache or neck pain is a presenting symptom in patients with spontaneous proximal aortic dissection. We report on a 53-year-old man with a history of migraine with aura, who was admitted to the hospital because of severe frontal headache and neck pain. An anterior chest pain lasting for 10 minutes the day before and a diastolic heart murmur suggested a proximal aortic dissection, which was confirmed by transesophageal echocardiography. Patients with proximal aortic dissection rarely have headache or neck pain, reflecting the low incidence of carotid artery involvement in this disease. However, differentiation between an isolated cervical artery dissection and a proximal aortic dissection extending to the carotid arteries is pivotal, since treatment options are vastly different.