This case shows a unicornuate uterus with associated left renal agenesis "Müllerian duct anomaly class II". There is no rudimentary horn, consistent with "type b".  There is associated hematosalpinx related to the drainage of the unicornuate uterus into the fallopian tube explaining primary amenorrhea. On initial clinical examination, there was a very small vagina, primarily concerning imperforate hymen or vaginal septum.