Abstract: Congenital pupillary-iris-lens membrane with goniodysgenesis (CPILMG) is a rare anterior segment disease, typically manifested as a pinpoint-sized pupil, continuously growing pupil membrane and goniodysgenesis.CPILMG usually has monocular disease and has no family heredity. Patients usually have albuginea and irisbulging that cover the pupil area, and are prone to have deprivation amblyopia and angle-closure glaucoma. The main treatment for CPILMG is surgical treatment. A small number of patients who has deep anterior chamber and spontaneous regression of pupil membrane can be followed up for observation. Present studies about CPILMG mainly focus on some case reports. It is easy to be misdiagnosed clinically with Axenfeld-Rieger syndrome (ARS) and persistent fetal vasculature (PFV). Learn more about the linical manifestations and differentiation of CPILMG will be helpful in clinical diagnosis and treatment. Research with larger sample size are expected to further investigate the different stages of CPLIMG and its developing process, its corresponding pathogenesis and treatment methods, so as to improve the understanding of the disease.(Int Rev Ophthalmol, 2021, 45： 415-419)

Key words: congenital eye disease/differential diagnosis, pupil membrane