A true presubclavian coarctation of the aorta with arch hypoplasia without major intracardiac anomaly can result in a surgical dilemma. Theoretically, one can avoid repair through median sternotomy using selective cerebral perfusion and its sequelae since no major intracardiac repair is needed. Repair through thoracotomy is technically challenging if arch reconstruction is required and precise surgical planning is required to avoid spinal hypoperfusion and to maintain cerebral perfusion. Moreover, inadequate repair often has resulted in hypoplastic aortic arch or residual coarctation on follow-up requiring future intervention. We have employed a modified surgical repair through thoracotomy avoiding cardiopulmonary bypass to address presubclavian coarctation of the aorta with diffuse arch hypoplasia with theoretically less chance for future stenosis.