Acute appendicitis has been reported to be present throughout the year, but some particular months are associated with higher incidences. The aim of this study was designed to review previous studies and analyze the current knowledge and controversies related to seasonal variability of acute appendicitis, to examine whether appendicitis has an annual seasonal pattern. A search of the MEDLINE databases for English language publications over the period from 1970 to 2012 was conducted. This list was extended by including also references from a recent review of appendicitis epidemiology. We selected the studies, which were published in an English-language peer-reviewed journal, did not have intervention, were conducted for at least one year and had the outcome of histologically-confirmed appendicitis and those which reported case data temporally. A total of 30 titles and abstracts were screened for eligibility. Of these, 11 studies from 8 countries conducted across from 1970 to 2012 met the selection criteria and contributed to the systematic review. A seasonal pattern of appendicitis with a mostly predominant peak is seen during the summer months in all the countries (except Nigeria and Turkey). Appendicitis is more common in summer months. The existence of a seasonal pattern in the onset of appendicitis may be due to exposure to air pollution, decreasing fiber diet and increase in the incidence of gastrointestinal infections in summer.