EEM syndrome is a rare condition characterised by ectodermal dysplasia, 
ectrodactyly and macular dystrophy. Additional abnormalities such as alopecia, 
cataract, absent eyebrows, and oligodontia may occur. We report two brothers and 
a sister born to consanguineous parents with EEM syndrome. EEM syndrome differs 
from other ectrodactly syndromes by the characteristic findings in the ocular 
fundus showing extensive retinochoroidal atrophy with diffuse retinal 
pigmentation and mild arteriolar attenuation at the posterior pole. In contrast 
to other ectrodactyly syndromes autosomal recessive inheritance is most likely.