Arteriovenous malformations (AVMs) are the most dangerous congenital vascular malformations. Intracranial AVMs occur in about 0.1 percent of the population and account for 1 to 2 percent of all strokes. The angioarchitecture of AVMs consists of direct arterial to venous connections without an intervening capillary network. The main symptom reported by half of all patients with arteriovenous malformations is an episodic headache. The headache is associated with nausea and vomiting and due to this resembles the hemicrania or cluster headache attacks. Although cerebral angiography is considered as the gold standard for the diagnosis, planned treatment and after treatment observation, AVMs are usually identified with advanced visual imaging techniques such as magnetic resonance imaging, computed tomography, and computed angiotomography. The treatment options consist of surgery, radiosurgery and endovascular embolization. We report a case of a 68-year-old man with giant intracranial arteriovenous malformation and right-sided hemicrania.