Unilateral agenesis of internal carotid artery (ICA) with intercavernous anastomosis is a rare congenital anomaly. We present a case of a 25-year-old female with 2-month history of holocranial headache. Neurological examination was unremarkable. Magnetic resonance imaging (MRI) with magnetic resonance angiography (MRA) showed absence of left ICA with an abnormal intercavernous vessel in the sella. Computed tomography (CT) showed absence of the left carotid canal. Doppler ultrasonography (USG) showed high resistance flow in the left common carotid artery (CCA). Since no hemorrhage or aneurysm was seen, patient was managed conservatively and is on regular follow-up. Based on our knowledge, this is the first case to demonstrate the features of unilateral agenesis of ICA with intercavernous anastomosis in X-ray, Doppler USG, CT, and MRI scans of the brain.