We report an unusual case of primary dystrophy of the retinal pigment epithelium (RPE) in which a vitelliform macular appearance was associated with reticular hyperpigmentation resembling the pattern dystrophies of the RPE. The entire evolution of the disease was observed and documented step-by-step during a 10-year follow-up. During the progressive change of the lesions morphologic characteristics of butterfly, reticular and macroreticular dystrophy as well as Best's macular dystrophy could be seen. Pathogenetic relationships between all of these dystrophies of the RPE can be supposed.