Persistence of proatlantal artery (PA) is a rare condition. More than 40 cases were described in the literature. Aneurysm may involve the PA itself in approximately 2% of cases, most arising from the internal carotid artery (ICA) side of PA. This case was particular because the PA showed a saccular aneurysm on the posterior wall, probably due to atherosclerosis disease and other alterations: plaque ulcerative of ICA, occlusion of left ICA, and aberrant right VA.