We report an unusual case of vitelliform macular dystrophy due to the presence of bilateral peripheral vitelliform lesions. Multiple forms involving posterior pole only and peripheral non specific lesions have already been described but this is the first case published, to our knowledge, showing typical vitelliform lesions in the periphery. These lesions developed in the same way as macular lesions, but they were complicated by a schisis in the left eye. In addition to other electrical and histopathological evidence, this case provides clinical evidence of the diffuse involvement of the retinal pigment epithelium in this disease. This involvement is linked to the accumulation of lipofuscin and granular substance, produced by photoreceptors. The macular predominance of vitelliform lesions can be partly explained by the metabolic and vascular particularities of the macula, but a genetic factor may be involved in the topographic determination of lesions. The development of a schisis on the edge of one of the described lesions encourages systematic search of peripheral lesions in cases of vitelliform macular dystrophy.