本文报告一例罕见的马凡氏综合征尸解病例,突出病变为肺动脉及其双侧肺内广泛分支夹层动脉瘤形成,主动脉瓣及肺动脉瓣巨大窗孔畸形,死于肺动脉夹层动脉瘤破裂及急性心包填塞。结合文献复习,认为马凡氏综合征时,肺动脉中膜囊性粘液变性是肺动脉夹层动脉瘤形成的基础;持续性肺动脉高压可能为其发生发展的条件。 This article reports a rare case of Marfan syndrome autopsy cases, prominent lesions of the pulmonary artery and its bilateral extensive bilateral dissection of the aneurysm, aortic valve and pulmonary valve huge window deformity, died of pulmonary artery dissection aneurysm rupture and Acute tamponade. Combined with the literature review, that the Marfan syndrome, the pulmonary cystic mucocutaneous degeneration is the basis for the formation of pulmonary artery aneurysm; persistent pulmonary hypertension may be its occurrence and development conditions.