A 68-year-old woman with a 5 yr history of chronic recurrent urticarial vasculitis with angioedema, refractory to conventional treatment including glucocorticosteroids, H1 and H2 blockers, nonsteroidal antiinflammatory agents, methotrexate, hydroxychloroquine, dapsone, azathioprine, intravenous gamma globulin, and plasma exchange, was successfully treated with interferon alpha 2a. The cause of the urticarial vasculitis remains unknown, although its onset followed a hepatitis A infection. This is the first report of the successful use of interferon alpha for chronic urticarial vasculitis/angioedema.