Since the first report of two pathogenic EPHB4 missense variants associated with LRFH,2 four additional cases of fetal hydrops (FH1:II.2, FH2:II.1, FH4:II.1, and FH5:II.2) and one case presenting with bilateral pleural effusions (FH3:II.7) with variants in EPHB4 have come to our attention (Fig. 1a–c). This evidence concerns the gene EPHB4 and hydrops fetalis.