We report two cases of Somali siblings with rare <i>CFTR</i> 896delT (c.764del; p.Ile255ThrfsX6; dbSNP rs2485020167; ClinVar ID 2682475; NM_000492.4:c.764del) homozygous variants who manifested classic CF lung and hepatic disease. Here, CFTR is linked to liver disorder.