Infantile nystagmus syndrome(INS)is a congenital pathological nystagmus characterized by binocular involuntary conjugative oscillation and reverse optokinetic nystagmus. This condition is often accompanied by amblyopia, strabismus, and torticollis, affecting the visual function of INS patients. As the cause of the disease is unclear and cannot be completely cured, early detection and appropriate intervention of INS should be carried out. Based on domestic and foreign researches of INS, in this paper, we summarize INS etiology and occurrence mechanism. Furthermore, to provide a reference for clinical application and future research directions of INS, we have systematically introduced the most recent INS examination and treatment methods, and highlight the problems in relevant clinical practice.