Abstract
Agenesis of the internal carotid artery (ICA) is an ultra-rare congenital anomaly. We report the case of an 11-year-old male patient who was referred to the pediatric neurology department for the assessment of left leg pain and intermittent, unspecified headaches for four years. On assessment, the left lower limb was wasted and shorter than the right one. Furthermore, he exhibited a complex collection of ocular diseases. Other examinations were unremarkable. Laboratory results were normal, and neuroimaging revealed a bilateral absence of the internal carotid artery. Nevertheless, the patient remained clinically stable, was not on any medications, and was regularly followed up for his comorbidities by a multidisciplinary team. This report highlights the various clinical manifestations of ICA agenesis and the need for a high clinical suspicion for early diagnosis and management. It also includes a review of the literature, highlighting the clinical aspects, embryological origin, and other reported cases.