Objective: To analyze the stimulating effect of axial length development on orbital volume development in patients (ages 12-60 years) with congenital microphthalmia. Methods: This retrospective cohort study included 43 patients (86 eyes) with congenital microphthalmia. Three-dimensional images of the orbit were generated from past computed tomography scans, and digital orbital volume and axial length measurements were taken. The patients were divided into four age groups for analyses. Paired t tests and one-way analysis of variance tests were used to compare orbital volume and axial length between the affected and unaffected eyes. Pearson correlation analyses and scatter plots were used to investigate the correlations between age, orbital volume, and axial length in the affected and unaffected eyes. Linear regression analysis was used to determine the association between orbital volume and axial length. Results: The mean orbital volume in the affected and unaffected eyes was 17.08 +/- 2.88 and 20.80 +/- 2.55 cm(3), respectively. The mean axis length in the affected and the unaffected groups was 12.73 +/- 3.54 and 23.84 +/- 1.43 mm, respectively. Significant differences were observed among orbital (t = 13.538, P < 0.001) volume and axial length (t = 21.339, P < 0.001) in the affected and the unaffected groups. There were no significant differences in affected orbital volume (F = 0.527, P > 0.05), unaffected orbital volume (F = 1.628, P > 0.05), affected axial length (F = 0.946, P > 0.05), and unaffected axial length (F = 2.217, P > 0.05) among the four age groups. According to the Pearson correlations, there were no significant correlations between age and affected volume, unaffected volume, affected axis, and unaffected axis (r = 0.095, 0.097, 0.084, and 0.022, respectively; all P > 0.05). Orbital volume was moderately correlated with axial length in the affected and unaffected groups (r = 0.470 and 0.410, respectively; both P < 0.01). Linear regression analysis revealed that a 1 mm change in axis length was associated with a 0.38 cm(3) and 0.73 cm(3) change in orbital volume in the affected and unaffected groups, respectively. Conclusions: In individuals ages 12 to 60 years old with congenital microphthalmia, the effect of axis length on the orbital volume growth of the affected eye is only half that of the unaffected eye. The eyeball, orbital tissue, and craniofacial development all play an important role in the growth of orbital volume.