To investigate the progression of myopic maculopathy (MM) and associated factors in highly myopic Chinese children.In this retrospective observational case series, biometric fundus features were morphometrically measured on photographs. Myopic maculopathy was defined as recommended by the Meta-analysis of Pathologic Myopia Study Group.The study included 274 children (mean age:11.7±2.5 years; mean refractive error:-7.66±1.87 diopters) with a mean follow-up of 4.9±1.2 years. MM progression was detected in 52 eyes (18.9%; 95% confidence interval [CI]:14.3%,23.7%). In multivariable analysis, MM progression was associated with a decrease in refractive error (odds ratio [OR]:0.72;95%CI:0.56,0.92;P<0.001) (i.e. higher myopization) and enlargement of parapapillary gamma zone (OR:7.68;95%CI:1.63,36.2;P=0.002). Incident peripapillary diffuse choroidal atrophy noted in 47 of 236 eyes (20.0%; 95%CI, 14.8%-25.2%), was correlated with a decrease in refractive error (OR:0.70;95%CI:0.54,0.92;P=0.009) (i.e., higher myopization) and greater gamma zone enlargement (OR:8.28;95%CI:1.33,51.7;P=0.02).Myopia in schoolchildren may have a considerable risk of progressing to MM. Enlargement of parapapillary gamma zone was a main independent risk factor.