Urticarial vasculitis is difficult to treat. We report here on a 40-year-old woman with a 16-year history of idiopathic hypocomplementemic urticarial vasculitis syndrome. Her disease had been resistant to treatment with H1- and H2-blockers, indomethacin, dapsone and interferon alpha but responded to > 25 mg/day prednisolone. Monotherapy with pentoxifylline was also of only minor benefit. Using a combination of dapsone (100 mg/day) and pentoxifylline (1,200 mg/day), we observed a gradual improvement resulting in a complete remission within 8 weeks. Complete control of symptoms could be maintained for 18 months without any serious side-effects. This type of treatment may be of benefit in other therapy-resistant cases of hypocomplementemic urticarial vasculitis syndrome, particularly in view of its excellent tolerance.