A 45-day-old female born at term after a normal pregnancy was referred for a urogenital malformation. Physical examination demonstrated normal vaginal introitus, urethra and labia minora. The upper part of the left labium presented as a 6 3 cm cylindrical mass and a clitoris-like structure with an accessory urethra located at the base. The anus was normally located (fig. 1). Blood, urine, renal function and hormone stimulation analyses were normal, and karyotype was 46,XX. Abdominal ultrasonography showed a normal right kidney and a hypoplastic ectopic left kidney with 2 bladders. The uterus and ovaries were normal. Retrograde cystourethrogram through the orthotopic urethra filled a bladder and induced grade II vesicoureteral reflux to the right kidney. That performed through the ectopic urethral meatus filled a second bladder located posterolaterally to the first one and induced vesicoureteral reflux to an ectopic left kidney. Cystoscopy confirmed 2 normal appearing bladders lined by normal mucosa, each receiving a corresponding ureter. Both urethras were also normal. At surgery 2 completely independent bladders were found together with an ectopic hypoplastic kidney, bicornuate uterus, 2 ovaries, a 36 cm long cecal prolongation resembling a mega-appendix and 1 vas deferens. The mega-appendix, left hypoplastic kidney, bladder, ectopic urethra and vas deferens were removed. No rudimentary gonadal tissue or testis was identified. Finally, the pubic mass was excised and vulvoplasty was performed. Pathological studies determined that the removed urethra was surrounded by prostatic tissue into which a blind ending vas deferens opened (fig. 2). Examination of the pubic mass revealed a chondrolipoma. Molecular studies for gender investigation were performed. Genomic DNA was extracted from paraffin blocks of kidney tissue, clitoris and vas deferens taken during surgery. DNA analysis was performed using polymerase chain reaction. Amplified products were electrophoresed on acrylamide gel and visualized using silver staining. Four of the loci tested on chromosomes 3, 9, 12 and 21 demonstrated different alleles, indicating the presence of 2 diploid genomes. Thus, haplotyping showed the heterozygotic origin of both duplicated elements. Fluorescent in situ hybridization was performed using an X centromere probe DX21 and Y cocktail probe but the results could not confirm 2 different genders. DISCUSSION