Mucinous appendiceal tumors include low-grade appendiceal mucinous neoplasm, high-grade appendiceal mucinous neoplasm, and mucinous adenocarcinoma. Nonmucinous adenocarcinomas are less frequent. Recent consensus guidelines and the latest edition of the World Health Organization classification will allow consistent use of agreed nomenclature. Accurate diagnosis is important not only for patient management but also to allow comparison of results between centers and tumor registries. Serrated polyps are the most common benign polyp in the appendix. They need to be distinguished from low-grade appendiceal mucinous neoplasm, which can also mimic other benign conditions. Goblet cell adenocarcinomas are a distinctive type of appendiceal neoplasm.