IntroductionOne rare cause of appendicitis is mucocele which may have a neoplastic or non-neoplastic source. Simple appendectomy or right hemicolectomy may be necessary, sometimes medication may be prescribed to manage symptoms. In this study, we reported a rare case with Mucocele of the Appendix managed by antibiotic therapy and simple appendectomy.Case PresentationA 60-year-old female with a seven-month history of right lower quadrant pain. A physical exam showed tenderness and rebound tenderness in the McBurney’s point. In her CT-Scan tubular mass measured 105 * 37 mm in RLQ is noted suggested for mucocele appendicitis. Appendectomy performed through McBurney incision and the pathology was mucocele of the appendix with mucin-secreting mucosal hyperplasia.ConclusionMalignancy of the appendix is extremely rare. Appendiceal tumors have a low prevalence; But due to the nature of these tumors, which can also be malignant, they should be given importance and careful follow-up is mandatory. As the Surgical community continues to learn more about this condition, it is essential that patients remain informed and proactive about this situation. With timely diagnosis and treatment, mucocele appendix can be successfully managed and treated.