Acute appendicitis, which requires immediate surgical intervention, is an important diagnosis in patients with acute abdomen. However, developmental abnormalities may interfere with the preoperative diagnosis and surgical treatment in some cases. Agenesis and atresia of the cecal vermiform appendix is an extremely rare clinical diagnosis. In addition, preoperative diagnosis may be difficult in some cases. Thus, diagnosis of the congenital absence of the vermiform appendix requires a thorough exploration of the retrocecal and ileocecal regions. A 59-year-old male was admitted from the emergency services with right lower abdominal pain. A celiotomy was performed with the suspicion of acute appendicitis. However, an atresia of the vermiform appendix was observed. The patient's appendix was thus removed. Pathological examination confirmed suppurative appendicitis. This case underlines the importance of the clinical entity for surgeons who may deal with a similar case. (C) 2013 Baishideng. All rights reserved.