Arteriovenous malformations of the head and neck pose a challenging therapeutic and reconstructive problem. In this report we describe a rare case of an arteriovenous malformation of the base of tongue. The patient, a young adult female, was treated with embolization followed by surgical resection using a lateral pharyngotomy approach. Reconstruction of the extensive base of tongue defect was accomplished using a radial forearm free flap. Currently, the patient is decannulated, articulates clearly, tolerates a normal diet, and is without recurrence. Our treatment approach is discussed in detail and compared with alternative techniques.