Progressive familial intrahepatic cholestasis type-2 (PFIC2), a severe liver disease which is familial, neonatal, progressive and often fatal which results from a mutation of ATP binding cassette subfamily B member 11 (ABCB11) gene which codes for an ABC transporter bile salt export pump (BSEP)1,2. This evidence concerns the gene ABCB11 and progressive familial intrahepatic cholestasis type 2.