The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome primarily affects the reproductive system of females. Although external genitalia is normal, the vagina and uterus are underdeveloped or absent due to this condition. Due to the absence of a uterus, affected individuals typically lack menstrual periods. Typically, the first noticeable sign of MRKH syndrome is the absence of menstruation by age 16 (primary amenorrhea). Individuals with MRKH syndrome have female chromosomes (46,  XX) and ovaries that function normally. Additionally, their breast and pubic hair development are normal. Although people with this condition are typically unable to carry a pregnancy, assisted reproduction may allow them to have children..