High myopia (HM) is characterized by a significant extension of the eye axis; it has emerged as a serious global public health issue recently. In addition to causing severe visual impairment, HM is associated with several problems that may compromise an individual's vision. Although genetic and environmental factors in HM have been extensively investigated, increasing evidence implicates the immune system and its microenvironment in its pathogenesis. In this review, we explore the complex interactions between cytokines, immune cells, and the eye environment to elucidate the complex processes controlling the immune response in HM. Furthermore, we investigated treatments modulating the immune response and alleviating the progression of HM and its complications. Through a review of the current relevant studies, we highlight the critical functions of the immune system in the multifactorial development of HM. With the evolving understanding of the immune system's involvement in HM, this review provides a valuable resource to clinicians and researchers to develop targeted interventions and personalized treatments for individuals with this vision-threatening condition.