Perineal fistula formation may be caused by rare anomalies of development of internal genital organs. We describe the case of the fistula formation due to functioning mullerian duct derivates in an adult male with normal genotype and phenotype. The MRI revealed the uterus presence with bilateral abdominal cryptorchism. To remove the fistula and for the oncology prevention we have performed laparoscopic hysterectomy, ovariectomy and fistula excision ad mass; the ovaries of the man were abnormally at the end of the fallopian tubes. In the long-term follow-up no signs of functioning fistula or any other pelvic pathology were discovered.