Abstract: Objective To summarize the clinical experiences of treatment of pulmonary artery dissection, in order to improve the diagnosis and treatment of the disease. Methods We retrospectively analyzed the clinical data of 4 patients including 3 males and 1 female with pulmonary artery dissection who were treated in Fu Wai Hospital between October 1996 and May 2009. Their age ranged from 17 to 45 years with an average age of 31 years. One patient with pulmonary artery dissection with aortic root aneurysm and chronic type Ⅱ aortic dissection was treated with Bentall’s surgery, total arch replacement and pulmonary artery angioplasty under deep hypothermic circulatory arrest. One patient with pulmonary artery dissection with patent ductus arteriosus (PDA) and severe pulmonary hypertension after a failed PDA transcatheter closure was treated with conservative strategy. For the one patient with pulmonary artery dissection with ventricular septal defect (VSD) and severe pulmonary arterial hypertension, VSD was repaired under cardiopulmonary bypass while left pulmonary artery dissection was left untreated. And conservative treatment was carried out for another patient with pulmonary artery dissection with VSD, PDA and Eisenmenger’s syndrome. Results Three patients recovered and 1 patient died of acute pericardial tamponade due to rupture of the dissection within 60 hours after onset of dissection. Followup was done in 3 cases with 1 lost. The followup time was 3 months and 4 years respectively. The New York Heart Association was class Ⅰ and Ⅱ. Conclusion Dissection of the pulmonary artery is a rare disease with a tendency of rupture and bad prognosis. Symptoms of pulmonary artery dissection are nonspecific, which can cause missed diagnosis. In patients with chronic pulmonary hypertension, the sudden seizure of chest pain, exertional dyspnea, and cyanosis, or worsened hemodynamics and cardiac shock may indicate pulmonary artery dissection which can be easily detected with echocardiography, CT scan and magnetic resonance imaging. Corrective surgery or conservative treatment can prevent rupture and possible death based on different causes of pulmonary artery dissection.