It is widely accepted that pathogenesis of childhood acute lymphoblastic leukemia (ALL) is related to the interplay between specific environmental exposure and inherited background. Major role of some members of the ATP-binding cassette (ABC) family of membrane transporters including MDR1 (ABCB1, P-glycoprotein) and breast cancer resistant protein (BCRP, ABCG2) is protection against environmental toxins. Here, we review several recent reports on potential association between single nucleotide polymorphisms (SNPs) in genes encoding for ABC transporters with predisposition to pediatric ALL.