Accessory spleen is defined as one, two, or three nodules of additional ectopic splenic parenchyma hung by a vascular pedicle generally near the spleen. Despite a relatively high frequency (from 10 to 30 % of the population based on autopsy studies), most accessory spleens are asymptomatic. Although cases of accessory spleen were clearly described in the literature, this perplexing diagnosis is often delayed and rarely made preoperatively. We report episodic recurrences of abdominal pain in a 66-year-old man attributed to iterative sub-torsions of an accessory spleen, as well as a comprehensive review of the literature.