Bi-allelic MYO5B gene variants, with the exclusion of bi-allelic variants that cause premature termination codons or the loss of the RAB11A-binding site at the carboxyl-terminus of the myosin Vb protein, can also cause isolated intrahepatic cholestasis with no or only mild intestinal symptoms [32,33,34]. The gene discussed is MYO5B; the disease is intrahepatic cholestasis.