A 5-day-old hornless goat was referred with dysuria since birth. The scrotum was absent, and a small penis-like structure was seen below the perineal raphe. On the laparotomy, the testicles were found near the inguinal ring- and attached to a uterus-like structure. On histological analysis, the uterus-like structure was blind-end. Germ cells were absent in the testis. The karyotype of this goat was 60, XX and the SRY gene was absent. The goat was homozygous for a DNA deletion responsible for the Polled Intersex Syndrome (PIS). To the authors' knowledge, this is the first report as the clinical case of the PIS-/- goat with urethral atresia.