We report a brother and sister with ectodermal dysplasia, ectrodactyly, and 
macular dystrophy (the EEM syndrome). Both children had abnormalities of the 
hands and the hair, and bilateral macular degeneration. The clinical picture in 
both is similar to, but less severe than, that described in the previously 
reported cases of this rare syndrome. Even though the parents are not related, 
they are both of Jewish Yemenite origin, and the possibility of a common 
ancestor cannot be ruled out. This would suggest autosomal recessive 
inheritance. The clinical picture in these patients suggests either variable 
expression or genetic heterogeneity in the EEM syndrome and further delineates 
the clinical and genetic spectrum of this condition.