Abstract
Nonimmune hydrops fetalis is a condition that may result as a cause of many underlying conditions and in around 15% of cases no cause could be identified. Hemophagocytic lymphohistiocytosis had been identified as a rare cause of nonimmune hydrops fetalis in the newborn period in few cases around the world. Here, we reported one case of nonimmune hydrops fetalis born for Saudi parents associated with Hemophagocytic lymphohistiocytosis presented at birth and was born at 32 weeks of gestation. Diagnosis was confirmed by bone marrow aspiration. She was treated with chemotherapy but did not respond to therapy and died. We suggest that Hemophagocytic lymphohistiocytosis should be seriously considered as a cause of nonimmune hydrops fetalis, if the later condition is associated with unexplained cytopenias, high ferritin level, and splenomegaly.