Probands from three consanguineous families with Lethal Congenital Contracture Syndrome 9 (LCCS9), a rare form of Severe Arthrogryposis Multiplex Congenita (AMC), were found to harbor different homozygous recessive mutations in the ADGRG6 coding sequence (Ravenscroft et al., 2015). Here, ADGRG6 is linked to arthrogryposis multiplex congenita.