Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome type 1 is a rare condition where the uterus and two-thirds of the vagina are not present. This condition occurs in females who have typical physical characteristics without other major anomalies. This form of MRKH syndrome is an isolated congenital condition, meaning that aside from the absence of the uterus and part of the vagina, people with this condition usually develop normal secondary sexual characteristics. It is one type within a broader group of reproductive tract malformations known by several names, including MRKH syndrome and Rokitansky sequence.

The main features of this condition include the congenital absence of the uterus and most of the vagina. People with this condition generally have normal external genitalia and secondary sexual characteristics, but may experience issues related to fertility and reproductive health.

Information about causes is currently limited for this condition.