Birdshot retinochoroidopathy is characterized by depigmented spots radiating from the optic disk in association with mild vitritis, retinal vasculitis, and involvement of the optic nerve head. In two patients, we traced the long-term course of uveitis with vitritis, retinal vasculitis, and papillitis that resulted in the typical cream-colored spots of birdshot retinochoroidopathy after seven and eight years, respectively, of follow-up. These observations suggest that in long-standing inflammation of the retinal vasculature and uveal tract, the HLA-A29 antigen should be assessed, because the development of typical lesions of birdshot retinochoroidopathy may be delayed in some patients.