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To examine our vascular anomalies team’s experience with tongue arteriovenous malformations (AVM) with specific emphasis on the spectrum on disease and surgical management.Retrospective chart review of 11 patients (10 female, 1 male), discovered from 1997 to 2005 with histological, radiographic, and clinical characteristics consistent with tongue AVM.Four patients displayed advanced disease with malformations involving the tongue, floor of mouth, and neck. Contributions from multiple feeding arteries were identified by angiography as each patient reported a prior history of surgical or embolic procedures. These lesions required preoperative embolization and extensive resection with complex reconstruction (mean operating time, 10.9 hr). One patient had evidence of recurrent disease (mean follow-up, 24.6 mo). In contrast, seven patients presented with discreet tongue malformations with a single feeding lingual artery. These patients reported no prior intervention, required only one resection (mean operating time, 2.8 hr), and have shown no evidence of recurrence (mean follow-up, 11 mo). Slight histologic differences between advanced versus focal tongue AVM were identified.This study suggests that tongue AVM can occur within a spectrum of disease with different clinical presentations, radiographic findings, and histology among patients with focal versus advanced lesions. Inadequate treatment is thought to contribute to collateral flow and disease progression in advanced AVM, making further management difficult. However, focal tongue AVM may represent early lesions that are more amenable to surgical management.