Herein, we report a compound defect of ABCC2 [c.2414delG, p.(Ile1489Gly), p.(Thr1490Pro) and p.(Ile1491Gln)] and UGT1A1 genes [c.-3279T>G, p.(Gly71Arg) and p.(Pro451Leu)] causing DJS and GS in a Han Chinese family, which are not exactly the same mutations as identified in reported dual-hereditary jaundice patients [7, 9, 10]. This evidence concerns the gene ABCC2 and Dubin-Johnson syndrome.