True umbilical cord knot is a common condition and often associated with perinatal morbidity and mortality. However, its association with hydrops fetalis is rarely observed. This report describes a case of hydrops fetalis associated with true knot of the umbilical cord. There were no other obvious causes for hydrops fetalis. On the basis of anatomic-pathological findings and review of the literature, the occurrence of hydrops due to umbilical cord true knot is proposed.

Keywords: Placenta, Nonimmune Hydrops Fetalis and Umbilical Cord