SUMMARY
This paper presents a 11 years old girl with an autossomal dominant reticular pattern dystrophy of the retinal pigment epitelium. The early diagnosis is possible and the evolution is slow. The visual
prognosis is good. Abnormal dark adaptation may be present. The electrophisiological study shows normal electroretinography and subnormal electro-oculography. The literature was reviewed and genetics aspects ware discussed.