In pediatric patients appendicitis is the most common cause of abdominal pain and surgery. Torsion of vermiform appendix is a rare cause, clinically indistinguishable from appendicitis with usually an intraoperative diagnosis. The first description of vermiform appendix torsion was made by Payne in 1918. Clinical presentation is similar to acute appendicitis. Preoperative investigations play a minimal role. Etiology of this condition is unclear, but is possible to distinguish a primary and a secondary torsion. We report a case of 5-years-old boy who presented with right lower quadrant abdominal pain. His clinical signs, symptoms and investigations mimicked an acute appendicitis. Intraoperatively we found a 720° appendix torsion on its base with its mesentery rotated in counter-clockwise direction. The appendix was gangrenous in appearance. A video-assisted trans-umbilical appendectomy was performed. We describe clinical presentation and management of this rare condition reviewing the literature.