Importance Pathologic myopia due to an excessive increase of axial length is associated with severe visual impairments. Systematic analyses to determine the rate of and the risk factors associated with the axial elongation in adults with high myopia based on long-term follow-up of a large population are needed. Objective To determine the risk factors associated with axial elongation in adults with high myopia. Design, setting, and participants This cohort study used the medical records of 43 201 patient visits in a single-hospital database that were collected from January 3, 2011, to December 28, 2018. A total of 15 745 medical records with the patients' sex, best-corrected visual acuity (BCVA), axial length, type of myopic maculopathy, and the presence or absence of choroidal neovascularization (CNV) were reviewed. Data were analyzed from April 3, 2019, to August 5, 2020. Main outcomes and measures Changes in the axial length at each examination were calculated. The significance of the associations between the annual increase of the axial length and age, sex, baseline axial length, types of myopic maculopathy, and a history of CNV was determined. Generalized linear mixed models were used to evaluate the strength of the risk factors associated with an increase of the axial length in high myopia. Results Among 1877 patients with 9161 visits included in the analysis, the mean (SD) age was 62.10 (12.92) years, and 1357 (72.30%) were women. The mean (SD) axial length was 29.66 (2.20) mm with a mean (SD) growth rate of 0.05 (0.24) mm/y. Among the 9161 visits, 7096 eyes (77.46%) had myopic maculopathy and 2477 eyes (27.04%) had CNV. The odds ratio for inducing a severe elongation of the axial length was 1.46 (95% CI, 1.38-1.55) for female sex, 0.44 (95% CI, 0.35-0.56) to 0.63 (95% CI, 13 0.50-0.78) for older than 40 years, 1.33 (95% CI, 1.15-1.54) for BCVA of less than 20/400, 1.67 (95% CI, 1.54-1.81) to 2.67 (95% CI, 2.46-2.88) for baseline axial length of 28.15 mm or greater, 1.06 (95% CI, 0.96-1.17) to 1.39 (95% CI, 1.24-1.55) for the presence of maculopathy, and 1.37 (95% CI, 1.29-1.47) for prior CNV. Conclusions and relevance This cohort study found continuing axial elongation in adults with high myopia. The risk factors for elongation do not appear to be modifiable, so prevention of myopia may be the best approach to reduce the incidence of pathologic myopia and its complications in the future.