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A 40-year-old man with a long-standing history of congenital arteriovenous malformation in his right forearm was seen with a large, pulsatile tumour in the hypothenar region extending with multiple tortuous vessels on the volar side of the forearm to the elbow crease. Preoperative Allen test and normal preoperative finger pressure measurement showed revascularization of the hand via radial artery. Ultrasonography, MRI and angiography described an arteriovenous malformation (AVM) type 4 Yakes, suggesting high risk for embolization. Surgical intervention with meticulous dissection and ulnar nerve release was performed in order to prevent important blood loss, distal ischemia and to preserve upper limb functionality. Surgical resection resulted in full recovery.