Urticarial vasculitis is characterized by pruritic, burning or painful, erythematous or edematous, circumscribed wheals with induration. It is most commonly idiopathic and it can occur in association with autoimmune disease, drug reactions, infections, or malignancy. Three forms of urticarial vasculitis has been described. These are normocomplementemic urticarial vasculitis, hypocomplementemic urticarial vasculitis, and hypocomplementemic urticarial vasculitis syndrome. We present a 17-year-old boy with urticarial vasculitis who has atypical lesions and venous thrombosis that related with Factor V Leiden mutation.