Urticarial vasculitis is an eruption of erythematous wheals that clinically resemble urticaria but histologically show changes of leukocytoklastic vasculitis. In association with connective tissue disease it is most commonly seen complicating Systemic lupus erythematous (SLE) and, less often, Sjogren's syndrome. Here, we report a 25-year-old woman who developed SLE in 1998. In May 2013 she presented with urticarial vasculitis; her skin biopsy was consistent with leukocytoclastic vasculitis. She also developed bilateral uveitis. She had most of the clinical and laboratory characteristics of hypocomplementic urticarial vasculitis syndrome (HUVS) which is difficult to be differentiated from SLE. She was treated with high-dose prednisone, Mycophenolate Mofetil (MMF), colchicine, and Dapsone but failed. We decided to give her Rituximab (RTX), her urticarial vasculitis and uveitis symptoms improved significantly. Unfortunately, later on she presented with severe discoid lupus. We started her on thalidomide and responded well. Our case highlights that Rituximab is a good option for severe refractory urticarial vasculitis and thalidomide is effective in treatment of discoid lupus erythematosus (DLE), and can be used safely in specialist rheumatological practice.