A 44-year-old non-smoking athletic Caucasian man with unremarkable medical history presented in the emergency room with unilateral right pressure-like headache. The pain had a rapid onset over 30 s and was rated 10/10 on a visual analogue scale. It was accompanied by nausea and right hemifacial hypoesthesia but not by vomiting, phonophobia, photophobia, tearing, rhinorrhea, nasal congestion, eye redness, visual impairment or motor deficit. Pain subsided within about 1 h after initiating analgesic treatment. The patient had suffered two similar headache episodes 3 and 6 days ago, rated 8/10 with spontaneous remission after 30 min. Right after the first episode, he felt a constant dull retrosternal pain rated 2/10, radiating to the back and aggravated by deep inspiration. He noticed that the subsequent episodes of hemicrania were preceded by exacerbation of the thoracic pain with radiation to the right side of the neck. A few hours after the second episode and for about 30 min, he saw flashing lights move slowly toward the midline in the right visual field, without fortification spectra and without ensuing headache. He denied any family history of migraine. Upon admission, the patient was in good general health with normal and symmetrical values of blood pressure on both arms and bradycardia (54 beats per minute). Neurological examination was unremarkable as was cardiovascular examination, including careful search for murmurs from large neck, thoracic and abdominal vessels. An electrocardiogram showed a normal sinus rhythm without signs of myocardial ischemia or infarction. Brain magnetic resonance imaging did not disclose any abnormality that could be related to the headache episodes. The concomitant presence of dull chest pain paved the way for suspecting aortic dissection. Contrast-enhanced cervico-thoraco-abdominal computerized tomography revealed type A aortic dissection sparing coronary arteries and supra-aortic vessels. Heart ultrasound showed an intact aortic valve. Testing for Marfan syndrome was negative. The patient underwent surgical replacement of the ascending aorta. No further episode of headache was reported after the intervention.