OBJECTIVE Developmental absence of the internal carotid artery (ICA) is a rare congenital anomaly that results from an insult to the third aortic arch or dorsal aorta during early embryogenesis. Patients are often asymptomatic and are diagnosed incidentally during imaging to investigate neurological complaints. METHODS We report a rare finding of an absent ICA during a workup of stroke in a middle-aged patient. RESULTS CT brain perfusion (CTP) and CT angiography (CTA) revealed the right middle cerebral artery (MCA) thrombotic stroke with the demonstration of contralateral left ICA absence. The patient showed spontaneous recovery, and no thrombolysis or neurointervention was considered. CONCLUSIONS This article highlights the importance of screening the head CT on bone window settings in case of the non-visualization of ICA to differentiate congenital absence from a steno-occlusive disease. It also illustrates the role of Magnetic resonance imaging (MRI) and MR Angiography (MRA) in demonstrating further possible vascular anomalies, structural brain malformations, and collateral circulation.