Mammalian UDP-glycosyltransferases (UGTs) are divided into four families: UGT1, UGT2, UGT3, and UGT8. UGT3 is the last of the gene families to be identified, and until relatively recently, little was known about the function of these enzymes. In this article, we present new analyses of the UGT3 family genes, including the structure of the UGT3A locus, interspecies sequence conservation, single nucleotide polymorphisms, and splice variants. We also review recently published work that has revealed that one member of this family, UGT3A1, has a unique enzymatic function: N-acetylglucosaminidation. Finally, we discuss the possible biological significance of the UGT3A enzymes.