We report on a female fetus with prenatally suspected hydrometrocolpos. Pos
tnatal evaluation additionally revealed ambiguous genitalia, anorectal atre
sia, vertebral segmentation anomalies and congenital intestinal aganglionos
is. Colostomy, was performed, but postoperative recovery, was complicated b
y pulmonary hypertension and renal failure, resulting in death at day 18. P
ostmortem examination furthermore revealed a small ventricular septal defec
t, as well as rectovaginal and urethrovaginal fistulae, causing massive dil
atation of the septated vagina (hydrocolpos). The possibility of an overlap
ping VACTERL and MURCS association is discussed.