We herein report a case of congenital bilateral hypoplasia of the internal carotid artery (ICA) that was diagnosed by bone window computed tomography (CT) and cerebral angiography. A 23-year-old woman presented with left dominant quadriplegia. Brain magnetic resonance imaging showed not only massive infarcts in the anterior circulation but also poor depiction of the bilateral ICAs. Bilateral carotid canals on bone window CT suggested hypoplasia. Cerebral angiography revealed narrowing of each ICA above its bifurcation, and the blood supply to the intercranial carotid systems developed from the vertebrobasilar system through the posterior communicating arteries and posterior cerebral arteries. We diagnosed the patient with congenital bilateral hypoplasia of the ICA based on bone CT and cerebral angiography findings. Performing both bone window CT and cerebral angiography can facilitate the diagnosis of congenital hypoplasia of the ICA.