The VLDL receptor (VLDLR) is a member of the LDL receptor family. The VLDLR was hypothesized to mediate fatty acid entry into peripheral tissues, on the basis of its expression in tissues that are active in fatty acid metabolism and its capacity to bind apolipoprotein-E-rich VLDL in vitro. This hypothesis initially proved difficult to confirm, because VLDLR-knockout mice were reported to display normal plasma lipid levels. Moreover, studies in VLDLR-knockout mice that were also deficient in a second LDL receptor family member, the apolipoprotein E receptor 2, indicated a role for the VLDLR in neuronal migration during brain development. However, in accordance with what the term VLDLR suggests, recent studies using VLDLR-deficient and transgenic mice have provided compelling evidence that the VLDLR does indeed play a role in VLDL-triglyceride metabolism, and that it is important for triglyceride storage in the adipocyte.