Arthrogryposis, renal dysfunction, and cholestasis (ARC) syndrome (MIM# 208085) is an autosomal recessive multisystem disorder caused by mutations in vacuolar protein sorting 33 homologue B (VPS33B; MIM# 608552) and VPS33B interacting protein, apical–basolateral polarity regulator (VIPAR; MIM# 613401) [Cullinane et al., 2010; Gissen et al., 2004]. The gene discussed is VPS33B; the disease is cholestasis.