Despite a low-incidence extracranial carotid artery aneurysm (ECAA) disease has important clinical repercussion that obliges understanding and knowledge of correct treatment. The 2 dominant etiologies are atherosclerotic degeneration and pseudoaneurysm. The natural history of ECAAs is understood. Neck pain, a pulsatile mass and central or peripheral neurological manifestations are the most common symptoms. Recommendations for diagnosis and treatment are not uniform and still under discussion, representing a challenge for clinicians. We discuss a case of 2.5 cm asymptomatic saccular atherosclerotic ECAA treated surgically in light of the most recent literature.