Developmental occlusive disease of the abdominal aorta and the renal and splanchnic arteries represent an unusual vascular condition. When unrecognized or untreated this disease is associated with premature death, usually from severe secondary hypertension as a consequence of renovascular stenotic lesions. Strong circumstantial evidence indicates that developmental abnormalities occurring during the fetal union of the two dorsal aortae account for most of the occlusive lesions affecting the abdominal aorta and its visceral branches in these patients. Complete arteriographic studies are necessary to confirm and accurately delineate the disease process. Surgical treatment, which often encompasses complex vascular reconstructive efforts, affords excellent results when carefully planned and executed.