Purpose: To examine 2-year progression rate and associated biometric changes in highly myopic eyes. Methods: This is a longitudinal, observational cohort study that included 657 participants aged 7 to 70 years with bilateral high myopia (≤−6.00 diopters [D]) and followed for 2 years. All participants underwent ocular biometry and cycloplegic refraction examinations. Main outcome measures were changes in spherical equivalent refraction (SE) and ocular biometry in the right eyes. Results: Mean age of participants was 21.6 ± 12.2 years. At baseline, mean SE was −9.82 ± 3.28 D and ocular biometric measurements were 27.40 ± 1.56 mm for axial length, 3.16 ± 0.27 mm for anterior chamber depth, 3.60 ± 0.35 mm for lens thickness, and 20.09 ± 1.50 mm for vitreous chamber depth. After 2 years of follow-up, there was a trend toward more myopia and greater axial elongation in all age groups. Younger participants (≤20 years) had significantly (P < 0.001) greater rates of myopic shift and axial elongation compared with older participants (>20 years). However, highly myopic adults aged 40 to 70 years continued to demonstrate refractive progression, particularly if they had extremely high myopia (≤−10.00 D). In the multiple regression analysis, each additional diopter of myopia at baseline was associated with a 11% higher risk of a >1.00-D/y myopic shift (odds ratio, 1.11; 95% confidence interval, 1.04–1.18; P = 0.002). Conclusions: Longitudinal data from this large Chinese cohort suggest that highly myopic eyes continue to progress in SE throughout life, with the greatest rates of progression observed in younger participants. Axial elongation rates appeared to stabilize after 20 years of age and were predominantly due to an increase in the vitreous chamber depth. Other risk factors for a myopic shift included a higher degree of myopic refraction at baseline.