A young woman presented with deteriorated visual acuity due to acute retinal vasculitis. The diagnosis of Behçet's disease was made on the basis of the ophthalmologic findings and recurrent oral and genital aphthous ulcerations. In the past, this young woman had been operated on several times because of progressive aseptic osteitis of the right clavicle. The actual examination revealed chronic osteitis, osteosclerosis and hyperostosis of the anterior chest wall, as well as severe acne papulopustulosa et conglobata. These findings resulted in an additional diagnosis of SAPHO syndrome. This report is the first description of Behçet's disease occurring in association with SAPHO syndrome, both fulfilling the diagnostic criteria.