Congenital coronary ostial stenosis or atresia (COSA) is a spectrum of rare developmental conditions with different pathophysiologic mechanisms and clinical implications. Although COSA includes various entities, these entities have two features in common. First the defect is congenital, although it may progress during pre-natal and post natal life. Second the developmental defect may cause ostial or proximal coronary obstruction (that is stenosis or atresia). Coronary ostial stenosis or atresia affects the left coronary (L-COSA) more frequently than it does the right coronary artery. Systemic Lupus Erythematosus (SLE) is not an uncommon disease in young female, but combination of congenital coronary ostial stenosis with systemic lupus erythematosus make the case very rarer. Here we presented A 17 years old girl got admitted to Bangabandhu Sheikh Mujib Medical University, Bangladesh for evaluation of on and off chest pain of CCS-III to CCS-IV for 1 day on 17 September 2019.