We report an extremely rare case of ruptured saccular aneurysm at the junction of the hypoplastic basilar and the persistent primitive trigeminal artery (PPTA). Three-dimensional computed tomography (3D-CT) angiography can delineate the aneurysm of the right persistent primitive trigeminal artery and the anatomy relative to the bone structure simultaneously. The neck of the aneurysm of the right PPTA was successfully clipped via left orbitozygomaticpterional craniotomy. Current diagnostic modalities and the surgical approaches for aneurysms of PPTA are discussed.