Beta zeolite modified with Sn in the framework (Sn-Beta) was synthesized and introduced as a heterogeneous catalyst for Baeyer–Villiger oxidations about twenty years ago. Since then, both syntheses strategies, characterization and understanding as well as applications with the material have developed significantly. Remarkably, Sn-Beta zeolite has been discovered to exhibit unprecedented high catalytic efficiency for the transformation of glucose to fructose (i.e., aldoses to ketoses) and lactic acid derivatives in both aqueous and alcoholic media, which has inspired an extensive interest to develop more facile and scalable syntheses routes and applications for sugars transformations. This review survey the progress made on both syntheses approaches of Sn-Beta and applications of the material within catalyzed transformations of sugar, including bottom-up and top-down syntheses and catalyzed isomerization, dehydration, and fragmentation of sugars.