The special nature of the anatomopathological structure and of the haemodynamic disturbances in congenital vascular defects causes certain peculiarities in their operative treatment. In our experience therapeutical strategy should be based on the following basic principles: active causal therapy combating pathogenesis, individual treatment, early operation, functionally radical operation, operation by stages and combined treatment. The numerous operative techniques are subdivided as follows according to surgical tactics: reconstructive operations, operations to remove the vascular defect, operations to reduce haemodynamic activity of the vascular defect and other nonhaemodynamic operations. Good postoperative results obtained after the use of the principles indicated above in a large number of patients treated by vascular surgeons in six different countries proved the validity of active and causal treatment of these severe diseases.