Abstract: Congenital nystagmus (CN) is an involuntary, rhythmic ocular oscillations that is often detected at birth or within 3 months of birth. The etiology of CN is complex and diverse. Till now, the pathogenesis of CN has not been entirely clear. CN is a difficult disease in the clinic of pediatric ophthalmology and strabismus, its treatment is difficult. In recent years, researchers have tried to improve the original surgical procedures in order to give patients better visual quality. Randomized, controlled, double-blind clinical studies have found that some drugs can reduce nystagmus intensity, prolong foveal fixation, and improve vision in patients. The genetic modes of CN are varied, and the pathogenesis of most of the CN is still unclear. Studies on model animals have revealed that the mechanism by which the FRMD7 gene, one of the pathogenic genes of X-linked CN,  is related to the loss of horizontal motion detection of direction selective retinal ganglion cells. This article reviews the recent progress research of CN in order to provide references for future clinical and basic studies. (Int Rev Ophthalmol, 2020, 44：  296-305)

Key words: congenital nystagmus, surgical treatment, drug therapy, genetics, pathogenesis