Background: Leiomyoma, also known as uterine fibroid is a benign tumour of the uterus. The size varies from a pin-head to a massive swelling occupying the whole of the abdominal cavity, depending on its site and duration. Huge uterine fibroid coexisting with umbilical hernia have been reported in the global literature. The aim of this report was to highlight a rare presentation of huge uterine fibroid with a large umbilical hernia, as well as the management and achievement of successful outcome, in a tertiary hospital in Port Harcourt, Nigeria. Case Presentation: A 43-year-old nulliparous woman who presented to the Emergency Department with features of intestinal obstruction, complicating a large umbilical hernia coexisting with a longstanding huge uterine fibroid. She had myomectomy and herniorrhaphy done, and the post-operative period was uneventful. Conclusion: This report not only showcases the unusual presentation of huge uterine fibroid with large umbilical hernia, but also highlight a picture of neglected surgical conditions in our practice. Action is needed from the political, administrative and professional personnel to forestall such occurrences among patients in our sub-region.