Patients with central areolar choroidal dystrophy are often difficult to diagnose because they are similar in fundus appearance to other retinal disorders. Nineteen patients with the diagnosis were studied in order to estimate the diagnostic and prognostic value of full-field electroretinography in this disorder. Our results showed that the cone b-wave amplitude in the ERG is decreased, that there is a progression in this decrease during a follow-up period of 5 years, and that the cone b-wave implicit time is prolonged. Central areolar choroidal dystrophy is considered to be primarily a choroidal disease. Our results indicate that it also affects most or all of the retinal cones. Further, our observations suggest that the disease is slowly progressive and that full-field electroretinography is of value in its early diagnosis.