Non-immune hydrops fetalis (NIHF) is a rare fetal condition with a very high mortality in spite of advances in prenatal diagnostic techniques, early detection, and individualized management. Despite advancement in fetal therapy and rapidly developing new knowledge about the aetiology and prenatal diagnosis, its management has remained controversial.This is a descriptive review ofNIHF.NIHF is a rare fetal condition that presents in an extremely acute manner with almost 90% mortality. Fetal cardiac anomalies are the most common cause and chromosome anomalies are the second-most-common cause. The worst prognosis was related to prematurity, severe hydrops, anaemia, cardiac malformations, chromosomal disorders and congenital infections. Fetal interventions includeboth medical and surgical modalities.NIHF is a rare condition with high prenatal mortality. The exact pathophysiology is still poorly understood. It is important to detect NIHF early, diagnose the underlying cause and institute appropriate treatment. There is need for autopsy of all fetuses or neonates who die from NIHF.