Hernia uterus inguinale associated with unicornuate uterus.

Hernia uterus inguinale, a condition in which endometrium and myometrium are found in an ectopic location in the inguinal canal, is a rare congenital abnormality in women. A woman with a normal number of chromosomes (46,XX) demonstrated the presence of a uterus, fallopian tube, and ovary in an inguinal hernia associated with a unicornuate uterus. This represents a unique abnormality in the spectrum of lateral fusion defects associated with müllerian ductal development, which normally proceeds as the right and left systems fuse and form the uterus, cervix, and four fifths of the vagina. In this patient, the left müllerian system apparently failed to fuse with the right, instead assuming a location within the patient's inguinal canal.