Arteriovenous malformations (AVMs) involving the upper limb constitute 10% of the total AVMs. In the upper limb, AVMs are more frequent in the hand than in the arm, being the hand one of the body's regions more frequently associated with AVMs, coming after the head and neck. The total prevalence of the upper limb AVMs remains unknown and there is currently no definitive consensus for the treatment of upper limb AVMs. The purpose of this study was to review the best evidence of the treatment for the upper limb AVMs and describe their clinical characteristics and diagnosis. The majority of patients with asymptomatic AVMs follows a conservative management. In the symptomatic patients, the treatment with surgery and or chemical embolization is beneficial. The amputation can be necessary in the case of life-threatening and massive AVMs, constituting the first step in patient's rehabilitation. Although the most common option for the management of symptomatic or functional AVMs is the embolo-sclerotherapy combined with surgery, different outcomes should be taken into account to plan the treatment, specially the presence of symptoms, bleeding and heart failure.