Mayer–Rokitansky–Küster–Hauser (MRKH) syndrome: A historical perspective

• MRKH syndrome is a congenital defect with variable genetic predisposition.
• We traced the evidence of MRKH syndrome back to 460 B.C.
• We reviewed the contributions of the authors in the eponym (MRKH).
• MRKH syndrome of Queen Amelia was a reason contributing to the dethronement of King Otto of Greece.
• We present evidence of primitive surgical techniques to rectify this condition.

Mayer–Rokitansky–Küster–Hauser (MRKH) syndrome is a congenital defect of the Müllerian ducts characterized by uterovaginal agenesis and underdeveloped female genital organs. This paper is a tribute to the contributors of this condition — August Franz Joseph Karl Mayer, Karl Freiherr von Rokitansky, Hermann Küster and Georges André Hauser. In addition to their contributions, we have discussed findings and reports of similar defects from other important scientists (Hippocrates, Albucasis, etc.) dating as far back as 460 B.C. We have also discussed the disease types and different classification systems including VCUAM and AFS/ASRM among others. Even with several surgical and non-surgical treatment options, there are still many questions that remain unanswered and very little is known about the etiology or genetic predisposition of this condition.