AbstractBackground of studyPolythelia is a cutaneous marker of congenital diseases of the kidneys. Lateral displacement of the nipples (e.g. laterally located to their respective midclavicular lines) is considered a further sign of renal disorders, particularly of bilateral renal hypoplasia, as reported by Fleisher in a detailed study on seven infants.SubjectWe observed a 45‐year‐old white man with his morphologically normal nipples displaced on the lateral aspects of the trunk without any other consistent clinical features such as accessory nipples or areolae, gonadal dysgenesis, face or car anomalies.Aims and methodsIn order to reveal congenital abnormalities of the kidneys and the urinary collecting systems the following investigations were undertaken: renal ultrasound, intravenous pyelography. abdomen CT and NMR scans, urethrocystography, transurethral cystoscopy, urinalysis, laboratory tests including karyotype, ECG and cardiovascular evaluation.ResultsInstrumental findings disclosed a hypoplastic left kidney in an ectopic site, located below the right kidney (cross type of renal ectopy) without any connection between their urinary‐ collecting systems. Complex malformations of the urinary tract such as an orthotopic narrow right ureteral orifice and ridge, a large‐sized diverticulum on the left wall of the bladder were also present. Moreover, a tumour on the upper pole of the right kidney, 20 mm in diameter, with a histological picture of renal‐cell carcinoma was detected.ConclusionsA lateral displacement of the nipples is a clue for the presence of renal hypoplasia both bilateral and unilateral. The significance of this association is unclear and its occurrence is probably under