Affected males show fetal or neonatal lethality.[2] Clinical findings of OSCS include macrocephaly, frontal bossing, wide nasal bridge, hearing difficulty, and abnormal palate.[3] Jenkins et al[4] have reported that mutations in the gene encoding WTX (Wilms tumor on the X chromosome; FAM123B or AMER1), a repressor for WNT signaling, are the cause of X-linked OSCS. This evidence concerns the gene AMER1 and Nephroblastoma.