A series of 58 patients of aortitis syndrome were analyzed from the standpoint of the pathogenesis and surgical treatment. Investigations of HLA revealed a high incidence of HLA-A9, BW52, MT1 and DR2 antigens with statistically significant differences in the chi-square test. These data indicate that a haplotype composed of A9, BW52, MT1 and DR2 is common in patients with aortitis syndrome and suggest that a genetic factor plays an important role on the pathogenesis of the disease. Experiences with the surgical treatment of various types of aortitis syndrome, such as operation for cerebrovascular insufficiency (7 cases), for hypertension produced by coarctation or renovascular stenosis (14 cases), for aortic regurgitation (2 cases), and for aneurysm (7 cases) were analyzed. Reconstructive surgery is difficult in many of them because of the complicated lesion produced by inflammatory changes. Attention should be paid to the selection of the most suitable time for operation, application of special surgical technique, and prevention of the complications to obtain favorable results.