Isolated extracranial internal carotid artery aneurysms (EICAAs) are rare, accounting for only about .4–4% of all peripheral artery aneurysms. We present a case of an EICAA in a young pregnant patient. To our knowledge, this is the first case being reported of a true EICAA during pregnancy in a multiparous woman. A 25-year-old pregnant patient presented with a pulsatile left neck swelling. Radiological investigations and subsequent surgery during the first trimester confirmed a true saccular left ICA with a maximum diameter of 3.5 cm. She was treated with aneurysm excision and primary anastomosis. She did not have any underlying diseases predisposing to an EICAA. She has been well for 3 years post procedure. It has been postulated that pregnancy could lead to aneurysm development through multiple mechanisms, including hemodynamic, hormonal, and other physiologic changes of pregnancy. Aggressive treatment with surgical intervention is advocated because of the risk of cerebral ischemic complications associated with non-operative treatment, and because satisfactory long-term outcomes can be achieved from surgery.