Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is an exceptionally rare condition. This article highlights its unusual presentation and the role of radiological imaging in the diagnosis and outlines an approach to management. A 17-year-old female presented to the hospital with symptoms typical of acute appendicitis. However, further evaluation revealed no menstrual bleeding, recurrent monthly abdominal pain, and the absence of a uterus on imaging. These findings raised suspicion for Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome. The presentation mimicking acute appendicitis in this context is an exceptionally rare occurrence. Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a rare condition that presents a considerable challenge in terms of diagnosis and management. A thorough clinical history, along with appropriate imaging and investigations, is essential for the timely identification of the syndrome and for guiding effective management.
Keywords: Absent uterus; Appendicitis; Mayer-Rokitansky-Küster-Hauser syndrome; Stump appendicitis.
© 2025 The Authors. Published by Elsevier Inc. on behalf of University of Washington.