McKusick-Kaufman syndrome is a rare autosomal recessive disease diagnosed by 
polydactyly, hydrometrocolpos, and congenital heart disease. We present an 
unusual laparotomy confirmed urogenital MRI finding (atretic vaginal pouch) in a 
3-month-old girl with McKusick-Kaufman syndrome. Up to our knowledge, this MR 
finding has not been reported in the literature yet.