We report on a 36-year-old male patient with a congenital arterio-venous malformation (type C) of the hand. Symptoms onset was three years ago with an ulcer on the tip of the left middle finger, with the fingers and the palm of the hand turning increasingly livid. The shunt-volume in the area of an arterio-venous fistula was 70 ml/min, there was also involvement of the third metacarpal bone. Increasing pain from the index to the ring fingers with no tendency of healing in the middle finger were indications for surgical intervention. In view of the diffuse expansion to all the tissue layers and the danger of peripheral circulatory disturbance and associated necrosis if embolisation was performed, the entire middle finger along with the third metacarpal bone was resected, and the index and the ring fingers were also amputated. Seven months postoperatively, the patient had no pain, had good grip function and was able to use his hand freely in his job as a labourer.