Drug release in a spatially and temporally controlled manner is highly desired in the field of drug delivery. Endogenous stimuli responsive nanocarriers provide significant advantages in maximizing the therapeutic efficacy and minimizing the side effects of loaded drugs. Biomarker-triggered release and site-specific delivery are the most commonly adopted strategies. In this review, the most recent advances in the field of endogenous stimuli-responsive drug delivery nanocarriers and their potential application in the treatment of various diseases are highlighted and discussed.