“…Since the first case of PEComa reported in 1996, cases involving various anatomic locations have been reported, such as gastrointestinal tract, 4 kidney, 12 pancreas, 13 liver, 14 ovary, 15 uterus 5 and so on. 16 , 17 According to the recent systematic reviews, 10 , 15 the most commonly affected organs were uterus (including uterine and cervix uteri) and colon, with approximately 65 and 20 cases reported online, respectively. Perirenal PEcoma are extremely rare, to date, only four cases have been reported and our case is the fifth one ( Table 1 ).…”