Hypoplasia of one or both internal carotid artery (ICA) is a rare congenital developmental abnormality. The early neurological presentation of this disorder is rare because many of these cases remain asymptomatic and go undetected due to the presence of collateral vessels. We describe a newborn that presented with seizures at 27 hours after birth. Extended ischemia of the right hemisfere was observed on computed tomography (CT), while the 3D MIP reconstruction showed hypoplasia of right internal carotid artery. After about 3 weeks, the rapid improvement of the newborn’s cerebral ultrasound and EEG allowed to discontinue corticosteroid and sedative therapy. The infant was discharged after 40 days of life in good clinical condition.