BACKGROUND Adamantiades-Behçet's disease is a multisystem disorder with recurrent oral and/or genital ulcerations, skin lesions, and ocular involvement. Eye involvement is a common manifestation that affects the patients' quality of life more than any other. Left untreated, it leads to blindness and often to loss of the eye through secondary complications such as phthisis or painful glaucoma. METHODS Case report of a 24-year-old patient of Turkish descent living in Germany since his birth was admitted to our Department of Ophthalmology in 1999 because of visual loss in his right eye. He could only perceive light in that eye and evidenced total retinal detachment. In the left eye he had 20/20 vision but showed signs of retinal vascular inflammation and secondary glaucoma. RESULTS Treatment with interferon alpha (9x10(6) I.U./3x week s.c.) and prednisolone (100 mg/d p.o.) led to complete regression of the acute inflammation within 2 weeks prior to operation. Vitrectomy was then successfully performed in the right eye under the therapy of interferon alpha (9x10(6) I.U./3x week s.c.) and 10 mg prednisolone p.o. The prednisolone therapy was stopped 1 week following operation. The failure of conservative glaucoma treatment necessitated trabeculectomy in the left eye. The patient has had no further recurrence for 4 years under monotherapy with interferon alpha (3x10(6) I.U./3x week s.c.). CONCLUSIONS Interferon alpha is a potent therapy for Adamantiades-Behçet's disease with ocular involvement. It also provides a basis for safe and reliable surgical interventions. There was no intra- or perioperative recurrence of inflammation, which is a common finding in these procedures.