A 27-year-old female visited outpatient clinic for the evaluation of palpitation and dyspnea on exertion. Echocardiographic examination including transthoracic and transesophageal echocardiography revealed coarctation of aorta, bicuspid aortic valve, mitral valve prolapse, and sealed-up ventricular septal defect. Further evaluation with computed tomography angiography revealed another combined congenital anomaly of left isomerism with polysplenism and interrupted inferior vena cava and pulmonary embolism with deep vein thrombosis. After corrective surgery for the coarctation, she received anticoagulation therapy for the treatment and the secondary prevention of pulmonary embolism.