Congenital defects of the reproductive system are associated with a number of adverse health consequences. The pathophysiology of these defects is thought to be müllerian abnormalities, but they can also be attributed to failure of cells to degenerate appropriately during embryogenesis. The management of these congenital defects is dependent on the type of defect present and the severity of presentation. Women with congenital defects of the reproductive system have physical as well as psychosocial issues that must be addressed, and the condition frequently creates medical challenges for health care providers.