McKusick-Kaufman syndrome is a rare, autosomal, recessive disorder characterized 
by hydrometrocolpos, post-axial polydactyly, and congenital heart disease. Less 
than one hundred cases have been reported in the English literature to date, 
mainly in the Amish population; sporadic cases have also been described. We 
present a case of an Arab Bedouin girl who presented with features resembling 
this syndrome.