Introduction: Appendiceal mucocele is a complex entity consisting of several different pathologies with a spectrum of clinical presentation. Case Report: We present a case of a 52-year-old male presented with a two-day history of abdominal pain. He was taken to the operating room for an open appendectomy and found to have a large mucocele of the appendix. Conclusion: We review the pathology as well as the different pathological conditions that may lead to an appendiceal mucocele.