Congenital AV shunting defects are not as rare as sometimes presumed, and they are four times more frequent in girls than in boys. Inadequate treatment can aggravate the patient's complaints by increasing the circulatory disturbance in the affected region. As a result, ischemic ulceration may develop. Special, usually interdisciplinary, treatment of such findings is mandatory. When AV communications are present, the combined treatment of surgical and nonsurgical techniques must be considered. Location and pathoanatomic type influence this choice. Clinical, functional, and hemodynamic parameters must also be evaluated in order to decide the optimal combination of therapeutic measures. Usually several phases of treatment are necessary.