Considering that Quist et al. first described the acute idiopathic scrotal edema (AISE) already in 1956, there are not many studies published in literature concerning the etiology, the development, and the progress of the disease since then. According to the literature the incidence of AISE is about 20%. Although it is an important differential diagnosis for acute scrotum, it remains extensively unknown. Therefore, AISE should be kept in mind by urologists, pediatric surgeons and pediatricians to avoid needless surgery or antibiotic therapy.