Hydrometrocolpos is a rare congenital disorder, which can present prenatally or after birth. It can be isolated or associated with other abnormalities. The abnormalities associated with hydrometrocolpos can be primary or secondary. Primary abnormalities arise from disruption of embryological processes while secondary abnormalities arise from the pressure effects of the distended vagina and uterus on adjacent organs. An analysis of autopsy findings of four cases of hydrometrocolpos at our center has shown a nonrandom association of certain congenital abnormalities. These abnormalities involve structures arising from the infraumbilical intraembryonic mesoderm. A primary developmental failure or secondary disruption of normally formed tissues results in this condition called lower mesodermal defects sequence. Identification of this condition is important in prognostication and counseling parents.