Appendiceal mucocele (AM) is a cystic dilatation of the appendiceal lumen filled by mucus inside, secondary to benign etiologies (hyperplasia and mucinous cystadenoma) or malignant (cystadenocarcinoma). This condition is uncommon, being reported in no more than 0.3% of appendectomies. Its size and clinical manifestations are extremely variable, allowing the pathological analysis of the surgical specimen etiologic diagnosis. We report a case of a giant AM secondary to a mucinous cystadenoma diagnosed in a 57-year-old man in the Pathology Unit of Hospital Hernán Henríquez Aravena of Temuco.