Introduction: We report a 4- and 1/2-month male child who had Nonrestrictive perimembranous VSD Left to Right shunt, with Tortuous and Hypoplastic arch with Retroesophageal Right Subclavian artery originating from Right Descending Aorta. CT angiography was done which revealed similar findings. PDA was connecting from right descending aorta to RPA. Materials & Methods: The the patient was taken for cardiac catheterisation. Catheter course was from Right Femoral artery to Descending Aorta to Transverse arch to Hypoplastic Tortuous arch to Ascending Aorta to LV. Significant gradient of 37mmHg was found between ascending and descending aorta. Hence patient was taken for surgery. VSD was closed with preformed oblong dacron patch with interrupted 5 -0 prolene pledgetted sutures along the posteroinferior margin & continous 5-0 sutures for rest of the margins, so as to dedicate the aorta across the VSD to the LV. Descending Aorta was mobilised so that it could be approximated to the ascending Aorta without tension and then it was anastomosed side to side to Ascending Aorta. Discussion: In type A interrupted left aortic arch, the arch interruption occurs distal to the origin of the left subclavian artery. In any of the 3 types, the right subclavian artery may arise normally or abnormally; the 2 most common abnormal sites are distal to the left subclavian artery (aberrant right subclavian artery) and from a right ductus arteriosus (isolated right subclavian artery).Thus in our case it was Type-I interruption with Right descending Aorta. Persistent fifth aortic arch (PFAA) has been subcategorised into three subtypes: (I) double-lumen aortic arch with both lamina patent; (II) atresia and interruption of the superior arch with patent inferior (persistent fifth) arch; and (III) systemic-to-pulmonary arterial connection arising proximal to the first brachiocephalic artery. Conclusion: In our case it was PFAA with (II) and (III) subtypes i.e. there was interruption of the superior arch with patent inferior (persistent fifth) arch joining Ascending and Descending Aorta; and (III) systemic-to-pulmonary arterial connection was established by PDA to RPA.