Perfectionism has long been recognised as a psychological factor that can enhance or interfere with the healthy adjustment of young students who are academically gifted. However, it is apparent from existing research that a wide range of methods have been adopted to study perfectionism in this population. To identify what is currently known about perfectionism among these students and what future work needs to be undertaken, a systematic review of existing research is required. The aim of our study was to provide a first such review. In doing so, we utilised the two-factor perfectionism model which differentiates between perfectionistic strivings (PS) and perfectionistic concerns (PC). A systematic literature search returned 36 studies examining perfectionism in young students identified as academically gifted that varied in study characteristics, methodological quality, and findings. Of these studies, 24 adopted a variable-based approach to examining perfectionism (i.e., examined PS and PC) and 12 adopted a group-based approach to examining perfectionism (i.e., examined groups with varying levels of PS and PC). The findings show that the distinction between PS and PC is extremely important. Specifically, while PC are likely to be uniformly debilitating for students who are academically gifted, PS are associated with more mixed outcomes. This is also the case when the two dimensions of perfectionism are considered in combination, with levels of PC being the key factor in determining the outcomes associated with perfectionism. Future research needs to build on the existing evidence base in a systematic fashion and prioritise longitudinal research and intervention studies.