In the literature it is possible to find many case reports of bladder exstrophy variants, although a thorough classification with all possible associated malformations is not yet available. On the basis of a rare case observed at their Department, the authors studied the embryology of these conditions and their associated malformations. The purpose of this study is to review the literature currently available and suggest a classification for bladder exstrophy variants. Despite the rarity of these variants, surgeons need to know all possible associated malformations in order to have the most complete and correct clinical picture of their patients.