Introduction: Torsion of the vermiform appendix is a rare intraoperative diagnosis. The clinical presentation is identical to that of acute appendicitis, with preoperative investigations playing a minimal role. The aetiology of the torsion is unclear, though it has been associated with fecoliths, lipomas and mucoceles of the appendix. Case Report: This case report describes a rare presentation of a four-year-old boy with a history and examination classical of acute appendicitis. Intraoperatively, a torsion of the vermiform appendix was found. The clinical presentation and operative intervention are reviewed. Conclusion: Torsion of the vermiform appendix is a rare cause of acute abdominal pain, mimicking acute appendicitis in presentation. Clinicians need to be aware of this intraoperative diagnosis and be able to manage it in the theatre.