Mucocele of the appendix is rare and represents only the tip of the iceberg of underlying benign and malignant pathological processes. Intraoperative diagnosis is also tricky because the inflammation of the appendix often hides the tumor. The preoperative diagnosis is essential to differentiate appendiceal mucocele from acute appendicitis as the treatment varies from open surgical versus laparoscopic surgical approach and for decreasing intraoperative and postoperative morbidity and mortality rate. We present three cases of appendiceal mucocele. The purpose of this paper is to make the physicians aware of the entity, its associations and the effect on management. This review will provide radiologic and pathologic correlation for the preoperative diagnosis of benign and malignant causative processes and differential diagnostic considerations.