Diabetes mellitus is among the most common chronic diseases worldwide. Infants of diabetic mothers are at increased risk of having congenital abnormalities. Tremendous progress has been achieved in the pregnancy care of diabetic women; however, the risk of birth defects associated with maternal diabetes still exists. These anomalies might arise in many organs and systems of the developing fetus. Many mechanisms have been implicated in the teratogenicity of maternal diabetes and it is critical to achieve good glycemic control before conception in women with diabetes. Neonatal clinicians must be able to identify patients at risk and recognize the signs of diabetic embryopathy. This article presents a review of congenital anomalies associated with maternal diabetes.