Atraumatic rupture of the spleen is an uncommon but important clinical entity. Unfortunately, the diagnosis is often missed due to the absence of any history of trauma. Various aspects of atraumatic rupture of the spleen, including those of 'pathologic' and 'spontaneous' rupture of the spleen, have been reviewed. Increased awareness of this condition would enhance early diagnosis and effective treatment. Further research is required to identify the possible risk factors associated with spontaneous rupture of the spleen.