Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) is a congenital condition affecting females, characterized by the absence or underdevelopment of the uterus and vagina. It occurs during fetal development and can lead to challenges in menstruation and fertility, although external genitalia typically appear normal. Individuals with MRKH may also experience associated anomalies in the kidneys or skeletal system. Diagnosis often occurs during adolescence when menstruation does not begin. Treatment options may include surgical interventions to create a functional vagina or assisted reproductive technologies for those wishing to conceive.