Sodium taurocholate cotransporting polypeptide deficiency (NTCPD) is an inborn error of bile acid metabolism caused by the solute carrier family 10 member 1 (SLC10A1) gene variants, which diminishes the enterohepatic circulation of bile acids by impairing the NTCP function as the primary transporter of conjugated bile salts from the plasma into hepatocytes^[1]. In 2015, Vaz et al. described the first NTCPD patient^[2]. Since then, sporadic cases with diverse genotypes have been reported in succession, but non of them were NTCPD accompanied with other autosomal diseases. Here, we present a rare pediatric case of NTCPD with α -Tthalassemia and Coffin-Siris syndrome (CSS).