Bilateral renal agenesis/hypoplasia/dysplasia is a lethal malformation in humans with an incidence of 1.3 per 10,000 live births. In the etiology of bilateral renal agenesis/hypoplasia/dysplasia, the genetic factor plays an important role. In addition to genetic factors, the etiology of bilateral renal agenesis/hypoplasia/dysplasia also involves the teratogenic effect of hyperglycemia on the embryo in mothers with insulin-dependent maternal diabetes. The purpose of this paper is to present a special case of bilateral renal agenesis/hypoplasia/dysplasia, which was successfully diagnosed prenatally by ultrasonography, confirmed and managed appropriately, so as to limit the incidence of serious, lethal congenital malformations.