INTRODUCTION: A clinical diagnosis of acute appendicitis does not necessarily imply only inflammatory changes found upon histopathological examination. Even though appendiceal tumors are very rare, accounting for less than 4% of all gastrointestinal tumors‚ they need to be taken into account and require the right choices regarding subsequent onco-surgical management. METHODS: 1036 appendectomies were performed and 38 (3.7%) rare appendiceal tumor cases were identified at the department of Surgery in Pilsen from 1 January 2008 to 1 October 2018. The following text presents the difficult nature of the initial diagnosis, current therapy and principles of follow-up care in this relatively rare group of patients. CONCLUSION: Appendiceal tumors are very rare. Depending on the histopathological type of tumor, it is necessary to choose adequate therapy and also provide proper follow up care.