Hypoplasia of the internal carotid artery and internal jugular vein ectasia are rare congenital abnormalities, whose diagnosis and treatment are not uniformly described. A 32-year-old neurologically asymptomatic woman with renal failure had a carotid artery duplex ultrasound scan as part of an evaluation for renal transplantation and was found to have bilateral internal carotid artery hypoplasia. Computed tomography angiography confirmed congenital bilateral internal carotid artery stenosis and left internal jugular vein ectasia. She had no neurologic deficits. She underwent antiplatelet treatment.