We report a case of bilateral aplasia of the internal carotid arteries. A 59-year-old man was admitted with generalized convulsions. Computed tomography and magnetic resonance imaging of the head showed normal findings. Three-dimensional computed tomography, angiography, and intra-arterial digital subtraction angiograms showed bilateral aplasia of the internal carotid arteries. The bilateral ophthalmic arteries were filled from the hyperplastic carotid arteries, and the anterior and middle cerebral arteries were filled from the basilar artery via the posterior communicating arteries. Associated with this bilateral aplasia of the internal carotid arteries, intracranial aneurysms and megadolicho-basilar anomaly were reported. Angiography is to be recommended if IC aplasia is suspected.