Objective:To study the pathogenesis, diagnosis and treatment  of vascular Ehlers-Danlos syndrome （EDS）.
Methods:Analysis of the clinical data of 2 patients with EDS and the EDS patients occurred in a fomily. The characteristics of pathogenesis, diagnosis and surgical treatment of EDS were reveiwed.
Results:One of the EDS patients had spontaneous rupture of aneurysm. At operation, the vessel was found to be fragile and the operative field had marked oozing of blood. Postoperative bleeding led to the patient death. Another case of  EDS underwent interventional treatment several times for thigh hematoma.but bleeding recurred quickly. Pedigree study of case 2 family showed autosomal dominant characteristics.
Conclusions:Vascular type EDS is a very rare kind of congenital disease.Its vascular complications often lead to disastrous consequences. Surgical therapy has a  high surgical complication and mortality rate.