Congenital carotid aneurysms are frequently found in the intracranial
arteries but rarely seen in extracranial part of the internal carotid
artery. A case of a congenital internal carotid artery aneurysm at the
skull base was treated in a 13-year-old female child. This case is be
ing presented for its rarity and also to highlight the ease of exposin
g the upper end of the aneurysm via lateral skull base approach. Infra
temporal fossa approach was adopted in this case. The carotid canal wa
s drilled off and the upper end of the aneurysm in the intrapetrous pa
rt was mobilized and vascular clip applied before dissection of the an
eurysm. Relevant literatures pertaining to this case are also reviewed
.