A kit for screening genetic liver diseases with high detection throughput, high sensitivity and strong specificity, the genetic liver diseases include progressive familial intrahepatic cholestasis, benign recurrent intrahepatic cholestasis, Congenital defects in bile acid synthesis, primary bile acid malabsorption, Dubin‑Johnson syndrome, hereditary hemochromatosis, alpha 1 antitrypsin deficiency, glycogen storage disease, autosomal recessive polycystic kidney disease, Budd Chiari syndrome , glycogen accumulation disease, etc. The genetic liver disease screening detects a total of 39 genes, namely ATP8B1, ABCB11, ABCB4, TJP2, NR1H4, HSD3B7, AKR1D1, CYP7B1, AMACR, ABCD3, SLC10A2, ABCC2, HFE, TFR2, SERPINA1, G6PC, SLC37A4, AGL, PYGL, SLC40A1, PKHD1, F5, GYS2, VIPAS39, SLC25A13, JAG1, NOTCH2, PHKA2, PHKB, PHKG2, PHKA1, ALAD, HAMP, HFE2, SMPD1, ATP7B, ABCA1, NPC2, NPC1; the kit includes targeted capture probes SEQ NO: 1 to SEQ NO: 722 for all exons of 39 genes. The invention is used for gene detection.