Congenital absence of the unilateral internal carotid artery (ICA) was found in a patient during MR imaging examination for right trigeminal neuralgia. Magnetic resonance angiography showed complete absence of the right ICA and a large tortuous basilar artery (BA). The source images revealed a deformed right trigeminal nerve resulting from compression by the BA. Computed tomography of the skull base showed absence of the right carotid canal, suggesting agenesis of the right ICA. Longstanding hemodynamic stress may have caused the BA to become extremely tortuous, resulting in the trigeminal neuralgia.