— Leiomyomas of the renal pelvis are rare benign tumours. Their bladder localization has been described in 160 cases, while their pelvic renal localization, according to our literature review, has been reported in just 6 cases. Of these, 5 underwent a nephroureterectomy and 1 conservative surgery, since the diagnostic preoperative examinations indicated urothelial disease. We report a case of fasciculated renal pelvis leiomyoma, in which the clinical aspect and the preoperative imaging indicated this fact and therefore the possibility of a conservative strategy.