Introduction: Metanephric adenoma is a rare benign kidney tumor. Patients with these tumors are usually asymptomatic, although polycythemia occurs in up 12% of cases. These masses are often described on diagnostic imaging as solid, single, well-defined, oval-shaped, unilateral lesions, located primarily in the renal medulla and without extrarenal involvement. These neoplasms are difficult to differentiate from malignant neoplasms of the upper urinary tract, so the definitive diagnosis is achieved by histopathology. Currently, the treatment of choice is radical nephrectomy. Case presentation: A 51-year-old woman from Bogotá (Colombia) was referred to the urology service of a tertiary care hospital due to bilateral lumbar pain of non-specific characteristics. At the time of consultation, the patient was asymptomatic. Renal and urinary tract ultrasound showed hydronephrosis and right renal mass. Computed tomography urography was requested, which revealed a lesion in the right renal pelvis with parenchymal invasion highly suggestive of high-risk upper urinary tract urothelial carcinoma, as well as adenopathies in the para-aortic lymph nodes. The patient underwent a radical nephroureterectomy with bladder cuff, which allowed establishing a final diagnosis of metanephric adenoma according to the histopathological study. Conclusions: Metanephric adenoma is a rare tumor that is difficult to diagnose through imaging, so it is necessary to explore additional tools to establish an accurate pre-surgical diagnosis that allows preserving the affected renal unit. Also, given their non-specificity, these tumors should be included in the differential diagnosis of lesions suggestive of upper tract urothelial carcinoma.