Pentalogy of Cantrell which usually comprises of anomalies of the ectodermal and mesodermal tissues is a very rare congenital condition which in the extreme of cases is incompatible with life. In this report a variant of the condition in a live newborn baby who presented to the University of Nigeria Teaching Hospital with abnormalities of the heart, urinary bladder and sternum is discussed. The aim of the report is to create an awareness and high index of suspicion amongst health professionals.