Parkes Weber Syndrome (PWS) is a congenital disorder characterized by the presence of arteriovenous malformations (AVMs) in upper or lower extremities. We herein present a 35 year-old male with PWS with complex AVMs in the right upper extremity; he had been previously treated with multiple sessions of vessel embolization, sclerotherapy and AVM resections. The patient presented to our clinic with two month history of progressive hand ischemia, digit necrosis and infection. Angiography was performed demonstrating numerous AVMs and filiform flow through the ulnar artery with poor opacification of arterial structures in the hand. Because of advanced ischemia, soft tissue infection and osteomyelitis, a distal forearm amputation was indicated. Hand threatening ischemia secondary to steal phenomenon associated to AVMs in PWS is rarely encountered and reported. This case illustrates a complex clinical presentation with advanced disease that required limb amputation.