Factor XIII (FXIII) is the final enzyme in the coagulation cascade that cross-links the fibrin monomers, thereby enhancing the clot strength. Congenital FXIII deficiency is rare and is inherited in an autosomal recessive manner. FXIII deficiency may lead to bleeding, delayed wound healing, hence increasing the risk for any surgical intervention. Literature on patients undergoing cardiac surgery with FXIII deficiency is rare. Cardiac surgery poses a major risk in this setting, as it involves heparinization while performing the surgery. This increases the risk of bleeding. In this case report, we present a patient undergoing coronary artery bypass grafting (CABG) surgery with FXIII deficiency. In this report, we focus on the perioperative management of FXIII deficiency, intraoperative surgical management, and postoperative surgical care in a patient who underwent CABG surgery with FXIII deficiency as opposed to standard CABG procedure.