Arteriovenous malformation (AVM) is a congenital disorder consisting of a connection between veins and arteries. Clinical findings may be headache, vertigo, epilepsy, hemiparesis, speech disturbance, and confusion. Involuntary movements have rarely been reported in AVMs. A 12-year-old male patient with a 3-year history of tremor in association with a thalamic AVM was reported. The lesion was localized to the left thalamochoroidal area. Neurologic examination showed hemiparesis, pyramidal signs, and dystonic tremor in the right limbs. In patients with dystonic tremor, thalamic lesions such as AVMs should be taken into consideration.