Persistent sciatic artery is a very rare congenital vascular malformation. Sciatic artery is the persistence of the axial artery in fetal life and connects the internal iliac arteries to the popliteotibial arteries. Femoral arterial hypoplasia causes the sciatic artery to persist and, therefore, becomes the dominant inflow to the lower extremity leading to the condition termed persistent sciatic artery. Persistent sciatic artery is a vascular structure which is prone to develop aneurysms and atherosclerotic stenosis. It is often characterized by aneurysm formation or thromboembolic complications causing lower extremity ischemia and neurological symptoms caused by compression of the aneurysm mass to the sciatic nerve. Angiography techniques can be used for the diagnosis and classification, while Doppler ultrasonography can be used for the diagnosis and follow-up. The decision on the treatment options depends on the overall condition of each patient, presenting symptoms, and classification of the disease. Endovascular interventions, which are the current treatment options, may be initially preferred with safe and effective results; however, careful follow-up is necessary.