SGLT1, an isoform of Na+−dependent glucose cotransporters, is localized at the apical plasma membrane in the epithelial cells of the small intestine and the kidney, where it plays a pivotal role in the absorption and reabsorption of sugars, respectively. Preferential localization of SGLT1 in the apical plasma membrane domain of these epithelial cells is crucial in the vectorial transfer of sugars. Mutations of SGLT1 gene have been found to be responsible for the congenital glucose galactose malabsorption syndrome. These mutant genes, when expressed in Xenopus oocytes, resulted in the alteration in their cellular localization. In this review, we focus on the molecular mechanism for the localization of SGLT1 in the polarized epithelial cells.