Alpha-tocopherol transfer protein (${alpha}$-TTP) is a liver cytosolic transport protein that faciliates ${alpha}$-tocopherol (${alpha}$-T) transfer into liver secreted plasma lipoproteins. Genetic defects in ${alpha}$-TTP, like dietary vitamin E deficiency, are associated with infertility, muscular weakness and neurological disorders. Both human and ${alpha}$-TTP deficient (${alpha}-TTP^{-/-}$) mice exhibit severe plasma and tissue vitamin E deficiency that can be attenuated by sufficient dietary ${alpha}$-T supplementations. In this review, we summarize the literature concerning studies utilizing the ${alpha}-TTP^{-/-}$ mice. Levels of vitamin E in the ${alpha}-TTP^{-/-}$ mice do not appear to be directly related to the amounts of dietary ${alpha}$-T or to the levels of ${alpha}$-TTP protein in tissues. The ${alpha}-TTP^{-/-}$ mice appear to present a good model for investigating the specific role of ${alpha}$-T in tissue vitamin E metabolism. Furthermore, ${alpha}-TTP^{-/-}$ mice appear to be useful to elucidate functions of ${alpha}$-TTP beyond its well recognized functions of transferring ${alpha}$-T from liver to plasma lipoprotein fractions.