Endometriosis is a common gynaecological condition characterised by ectopic endometrial tissue growth beyond the uterine cavity. Urinary tract endometriosis represents only 1.2% of all cases, with renal endometriosis accounting for less than 1% of urinary tract involvement. An asymptomatic, 49-year-old, perimenopausal Irish female was found to have an incidental mass at the upper pole of the right kidney on imaging studies. Histological analysis was recommended to outrule a renal malignancy and an open radical nephrectomy was performed following multidisciplinary input. Histological analysis surprisingly revealed the presence of endometriosis and endosalpingiosis, accompanied by a signi cant smooth muscle component, which mimicked a renal neoplasm. The aetiology of renal endometriosis remains unclear. However, given the lack of a history of endometriosis and the absence of other foci of disease in this patient, together with the smooth muscle predominant phenotype, this may represent a case of renomullerian fusion or endometrial displacement during gestational development. Keywords Endometriosis, smooth muscle, reno-mullerian fusion, malignancy