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To perform a longitudinal analysis on the association of corneal haze with intraocular pressure (IOP) in eyes with primary congenital glaucoma (PCG) over 3 years.Charts of all patients diagnosed with glaucoma of childhood from 2002 to 2012 at our institution were retrospectively reviewed. Inclusion criteria were age 18 years and below, plus elevated IOP or characteristic clinical signs. Exclusion criteria were eyes with secondary glaucoma or corneal haze not from PCG and patients with prior ocular surgery or incomplete follow-up.Of 79 eyes with childhood glaucoma during this period, 36 eyes had PCG [25 patients; 15 male (60.0%), 14 bilateral (56.0%)]. Eighteen eyes (13 patients) presented with corneal haze, whereas 18 eyes (12 patients) did not. Eyes with haze were diagnosed at a younger age than eyes without haze (0.79 vs. 5.2 y, P<0.02). During year 1, eyes with haze underwent significantly more IOP-lowering procedures and used significantly fewer IOP-lowering medications. Multivariate analysis revealed that corneal haze increased IOP by 4.63 mm Hg when controlling for treatment over time (P<0.01). Eyes with haze had lower survival curves and a failure hazard of 1.3 times that of eyes without haze. These eyes had a lower proportion of qualified successes than eyes without haze at year 1 (P<0.05) but this was reversed at year 3 (P<0.02).Eyes with PCG-related corneal haze generally presented more severely than did those without haze, but postmanagement outcomes may be similar to those in eyes without haze.