BACKGROUND: Schistosomiasis is a chronic granulomatous inflammation that affects many systems in the body including the gastrointestinal tract. This study was carried out by reviewing all cases of schistosomal appendicitis, and documents any association with acute appendicitis. OBJECTIVE: To review all cases of schistosomal appendicitis and document any possible asspciation with acute appendicitis. DESIGN: A retrospective study. SETTING: Obafemi Awolowo University Teaching Hospitals Complex, Ile-Ife, Nigeria, 1991 to 2004. SUBJECTS: Eight hundred and forty three specimen of appendicectomy were reviewed. Thirty five of them were diagnosed as schistosomal appendicitis. RESULTS: The involvement of the vermiform appendix by schistosomiasis found in 35/843 (4.2%) cases of all the appendicectomy specimen received in our histopathology laboratory between 1991 and 2004 shows that 23 of the cases (65.7%) had histologically proven acute appendicitis while the remaining 12 cases (34.3%) were schistosomiasis without active inflammation. The appendiceal wall oviposition is associated with submucosal fibrosis, narrowing of the lumen and subsequent acute suppurative inflammation in 17 cases while there were active granulomas with tissue eosinophilia in six cases. CONCLUSION: This finding has demonstrated that though the frequency of appendix involvement is low considering the endemicity of schistosomiasis in our environment, however acute appendicitis may be caused by schistosomiasis.