AIM: Histopathological examination of the appendix after appendectomy is routinely performed. The object of this systematic review is to determine whether routine histopathological examination of the appendix is justified. METHOD: PubMed, EMBASE, Web of Science and the Cochrane library were searched without language restriction up to 1 October 2009. All articles that reported on the incidence of histopathologically proven aberrant appendiceal pathology were included. RESULTS: Nineteen case series reported the incidence of a benign neoplasm [0.5%, weighted mean (WM)], malignant neoplasm (0.2%, WM) and other pathology (0–14%). Nine articles reported the sensitivity of the intra-operative findings to detect aberrant diagnoses. Parasitic infection was detected in 0–19%, endometriosis in 0% and granulomatosis in 0–11% of cases. Five articles addressed the consequences of aberrant pathology. Most patients with parasite infection, granulomatosis and malignant neoplasms underwent additional investigation or treatment, in contrast to patients with a benign neoplasm. CONCLUSION: The incidence of unexpected findings in appendectomy specimens is low and the intra-operative diagnosis alone appears insufficient for identifying unexpected disease. The benefit of histopathology is studied inadequately. From the present available evidence, routine histopathology cannot be judged as useless.