To evaluate visual field changes in primary congenital glaucoma (PCG) with retinal nerve fiber layer thickness on optical coherence tomography. In this cross-sectional, observational study, consecutive PCG children who underwent combined trabeculotomy with trabeculectomy and on regular follow-up were enrolled. All patients were aged over four years and co-operative for RNFL OCT and visual field examination. Perimetry was done on Humphrey visual field (HVF) analyzer using 30-2 and 10-2 SITA standard algorithms as appropriate. If a reliable automated perimetry was not feasible, kinetic perimetry was done. The following were noted at baseline and every follow-up: age, sex, visual acuity, intraocular pressure (IOP), cup-disc ratio (CDR), corneal diameters, refraction, any topical antiglaucoma medications, surgeries underwent, age at surgery and duration between surgery and final examination. Forty-eight eyes of 34 children operated for PCG and 19 eyes of 17 controls were analyzed. A statistically significant thinner average RNFL thickness of 87.2 ± 28 μm was noted in PCG eyes as compared to controls with 100.6 ± 7.2 μm (P = 0.04). The mean cup-disc area ratio on OCT in PCG eyes was 0.43 ± 0.2 (0.02-0.93) and in control eyes was 0.23 ± 0.07 (0.1-0.4) (P < 0.001). On RNFL OCT, there was significant focal RNFL loss in temporal superior (P = 0.003), nasal inferior (P = 0.037) and temporal inferior (P < 0.001) quadrants compared to controls. Among PCG eyes, 20/48 eyes (41.7%), had definitive, reproducible glaucomatous VF defects. Mean baseline IOP in PCG eyes with VF defect was 28.7 ± 5.7 mmHg and in eyes with normal VF was 24.6 ± 5.9 mmHg (P = 0.03). On univariate regression analysis, higher baseline IOP was significantly associated with both RNFL loss (odds ratio (OR): -2.17) and VF defects (OR: 3.35). Fluctuation in follow-up IOP (OR: 3.33) was also significantly associated with the presence of VF defects. On multivariable regression analysis maximum, IOP was significantly associated with RNFL loss and VF defects. Peripapillary RNFL thickness could be used to identify PCG eyes having visual field loss and possibly poor visual function from PCG eyes without visual field defects. Baseline and follow-up IOP, significantly correlated with RNFL thickness in PCG eyes.