The vitelliform macular lesion is usually observed in Best's disease and the adult type of foveomacular dystrophy. We describe a rare case of macular vitelliform lesion in association with basal laminar drusen. The patient has been followed at the Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo for the last four years. The differential diagnosis, clinical evolution, pathogenesis and treatment of this rare condition are discussed.