We report an 8-year-old girl presenting with a parotid gland enlargement since her birth. All clinical and radiological findings indicate a vascular abnormality more likely an arteriovenous malformation. Partial parotidectomy was performed for cosmetic reasons by preserving all branches of facial nerve despite a very bloody operation. The pathology report indicated arteriovenous malformation surrounding adenoid cystic carcinoma with a diameter of 12 mm with intact surgical margins. (C) 2012 Published by Elsevier Ireland Ltd.