Type 1 diabetes is an autoimmune disease due to the destruction of insulin-producing pancreatic beta cells. Natural Killer T (NKT) cells are a T-cell subset that links the innate and adaptive immune systems. NKT cells play a key regulatory role in type 1 diabetes. The absence of NKT cells correlates with exacerbation of type 1 diabetes, whereas an increased frequency and/or activation of NKT cells prevents beta-cell autoimmunity. Various mechanisms are involved in the protective effect of NKT cells. The goal is now to translate knowledge gained from mouse models into human therapeutics.