Studies have suggested that LCCS is an autosomal recessive disorder causally linked to 11 genes, including GLE1, ERBB3, PIP5K1C, MYBPC1, DNM2, ZBTB42, CNTNAP1, ADCY6, ADGRG6, NEK9 and GLDN. Among them, LCCS1 (MIM #253,310), LCCS7 (MIM #607,598) and LCCS11 (MIM #617,194) have been relatively frequent reported in the literature [1–6]. This evidence concerns the gene GLE1 and lethal congenital contracture syndrome.