Li et al. identified an in-frame insertion in EPHB4 in a family with a history of fetal hydrops and lymphovenous dysfunction characterized by edema of the lower extremities, venous stasis, and variable chylous effusions.4 This work confirmed the important role of EPHB4 in the function and development of the lymphatic system and the association of EPHB4 variants with fetal hydrops of lymphovenous origin. This evidence concerns the gene EPHB4 and hydrops fetalis.