Abstract: Objective To investigate the clinical features, diagnosis, treatment and prognosis of quadricuspid pulmonic valve malformation. Methods The clinical data, diagnosis and treatment process of a patient with quadricuspid pulmonic valve malformation were reviewed, and the related literatures at home and abroad were analyzed. Results The patient's preoperative examination showed atrial fibrillation; mitral valve calcification with stenosis and insufficiency; main pulmonary artery dilatation with pulmonary insufficiency; and tricuspid valve insufficiency. After complete examination, mitral valve replacement, pulmonary valve replacement, tricuspid valve repair and atrial fibrillation ablation were performed at the same time. Two dimensional echocardiogram showed no obvious stenosis and regurgitation of each valve after operation. There was no deterioration in cardiac function during the 1-year follow-up. Conclusion Quadricuspid pulmonic valve malformation is a rare congenital cardiac structural abnormality, which is prone to secondary valve and macrovascular disease in the later stage. Transthoracic valve replacement is still the mainstay of treatment. The condition of the pulmonic valve and the presence of deformities should be carefully evaluated before autologous pulmonary valve transplantation.