Recently, many advances have been made in the study of sexual differen
tiation, including the discoveries of the gene for antimullerian hormo
ne as well as the gene for its receptor. However, the etiology of the
clinical syndrome of mullerian agenesis remains elusive. We hypothesiz
e that activating mutations of either the antimullerian hormone gene o
r its receptor gene may cause mullerian duet regression in a genetic f
emale during embryogenesis. This clinical commentary discusses the cur
rent management of the syndrome including the Abbe-McIndoe procedure,
the most commonly used method of surgical correction, rind the Frank v
aginal dilation method, the most common nonsurgical method of correcti
on. (C) 1997 by The American College of Obstetricians and Gynecologist
s.