Appendicectomy is one of the most commonly performed surgical procedures. Appendiceal mucocele is a relative rare disease, but appropriate management is critical. Indeed, the intact removal of a mucocele represents a curative treatment; conversely, a rupture may result in the spread of epithelial cells throughout the peritoneal cavity (pseudomyxoma peritonei). We report a case of a 61-year-old woman, admitted to our department, who underwent resection of an appendiceal mucocele, focusing, in the discussion, on the clinical and surgical management of this disease.