Extracranial carotid artery aneurysms are rare. The most common location is the common carotid artery near the bifurcation. The mid to distal internal carotid artery is the second most common location. We are reporting the case of a 64-year-old woman who was admitted to our department for management of an asymptomatic left internal carotid artery aneurysm. Physical examination revealed a pulsatile mass, and imaging confirmed the aneurysm diagnosis. Computed tomography angiography detailed a 28mm×3cm×6cm aneurysm of the left cervical internal carotid artery with tortuous outflow the aneurysm sac. Open repair was undertaken. Exposure with incision anterior to the sternocleidomastoid was performed although extended more superiorly than usual because of the distal aneurysm location. After carotid clamping, the aneurysm was resected and an end-to-end anastomosis with prosthesis was performed. After closure, the patient was extubated demonstrating baseline neurologic function. Histologic examination of the arterial wall confirmed the diagnosis of fibromuscular dysplasia.

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