Objective To explore the characteristics in clinic and the value of Echo diagnosis of TOF with SIE. Methods A young male patient was diagnosed TOF many years ago. Later, no obvious factors appear on the left side of the body lack of power, and elbow can,t activities. Recently, he was no obvious history of fever. Echocardiography (echo) diagnosis: CHD TOF, the abnormal hyperechogenicity on aortic valve. Results Postoperative Diagnosis: CHD TOF merger SIE, aorgic valve with vegetations. Pathological Report: Fibrous tissue hyperplasia of the aortic valve, hyaline degeneration and myxoid degeneration. Conform to the change of the SIE. Conclusion TOF with SIE is very rare. When sometimes SIE has not typical clinical symptoms, the positive rate of blood culture is also low. Echocardiography examination can give a clear early and an important diagnosis，which can guide clinical early diagnosis, provide important information to select a treatment and evaluate prognosis.