Arteriovenous malformation (AVM) is defined as a convolute of abnormally connected arteries and veins, where the capillary bed is missing. Intracranial cerebral AVMs are 20 times more common than extracerebral. Focal lingual artery AVMs, especially at carotid bifurcation are rarely reported in the literature. Magnetic resonance imaging, angiography, digital subtraction angiography are the current modalities of diagnosis. Surgical resection can be an effective treatment for vascular malformation; however, the potential for large volume blood loss poses a challenge. In addition, the inability to identify the surgical margins of the nidus can result in recurrence. Present case was diagnosed based on radiological imaging as lingual AVM at the bifurcation of carotid artery. The paper discusses the case of a AVM of the tongue in a 23-year-old female patient at the level of carotid bifurcation. The paper further discusses the various imaging modalities used to diagnose the condition and review the latest literature on the current management concepts. Utmost care needs to be exercised when AVMs are suspected. Radiological investigations are must to know the location and extent of the lesion before attempting surgical intervention like biopsy or excision, which poses life-threatening complications like massive hemorrhage and damage to vital structures.
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