Herein, we identified two novel mutations in COL3A1 gene associated with vEDS, and added pathogenic evidences of two mutations in COL3A1 gene, four gene mutations of non-vascular types underlying EDS in four patients, and one gene mutation associated with collagen synthesis in one patient, all in patients presenting with pulmonary hemorrhage. This evidence concerns the gene COL3A1 and Ehlers-Danlos syndrome.