A 56-year-old man had aneurysms of the right subclavian artery and cerebral artery in association with congenital absence of the right internal carotid artery. The aneurysm of the subclavian artery was successfully surgically repaired through a partial sternotomy. Congenital absence of the internal carotid artery is rare vascular anomaly. This anomaly contributes to the occurrence of intracranial aneurysms. However, aneurysm of subclavian artery associated with congenital absence of the internal artery is very rare. This is the 3rd case reported in the literature.