We report on a Brazilian kindred in which two sibs presented with the complete 
form of EEM (ectodermal dysplasia, ectrodactyly, and macular dystrophy) syndrome 
with hypotrichosis, dental anomalies, syndactyly, and retinal changes with 
prominent pigmentation in the posterior pole of the retina. In this family, we 
also observed another sib with syndactyly, as well as a first cousin with 
ectrodactyly. A 10-year follow-up demonstrated gradually decreasing visual 
acuity and progression of retinal degenerative anomalies.