Congenital anomalies of the inferior vena cava are rare but if unidentified may create a risk for complications during surgery. In cardiac surgery, identifying such anomalies is crucial as they may alter the normal conduct of cardiopulmonary bypass. Herein, we describe a case of a 78-year-old women with an anomalous inferior vena cava draining into the superior vena cava, who was referred for surgical management of severe mitral regurgitation. We describe the clinical implication of the inferior vena cava anomality and the surgical strategies used to obtain adequate surgical exposure and venous drainage for cardiopulmonary bypass in a patient who underwent a mitral and tricuspid annuloplasty, bi-atrial MAZE procedure and a left atrial appendage closure.