The purpose of this report is to describe a case of nonimmune hydrops fetalis that resulted from an unusual congenital heart defect, premature closure of the ductus arteriosus. In this fetus, the ductal closure was not associated with other heart defects such as tetralogy of Fallot or truncus arteriosus, nor was it related to maternal use of nonsteroidal antiinflammatory agents. Despite adequate digitalization of the mother, the fetus died of congestive heart failure at 29 weeks of gestation. Autopsy confirmed stricture of the ductus in association with enlargement of the foramen ovale and marked dilation of the right atrium and main pulmonary artery.