Objectives To provide in vivo measurements of anterior chamber angle structures and their relationship with age as evaluated by high-frequency ultrasound biomicroscopy (UBM) in patients with primary congenital glaucoma (PCG) Methods High-frequency UBM was done for 51 PCG eyes from 40 patients (aged from 3 to 96 months) and 11 unaffected contralateral eyes. Parameters, including the proportion of observable abnormal tissue membrane and Schlemm's canal, the largest cross-sectional area (CSA) of Schlemm's canal (SC), SC meridional diameter, trabecular-iris angle (TIA), trabecular meshwork (TM) thickness, iris thickness, ciliary process length, and corneal limbus thickness were compared between the two groups and their relationship with age was explored in PCG eyes. Results Abnormal tissue membrane was detected in 27.5% of PCG eyes and none in unaffected eyes. SC was observed in 73.1% of PGC eyes compared to 100% in unaffected eyes (P<0.001). The largest CSA of SC, SC meridional diameter, iris thickness, and corneal limbus thickness were all significantly smaller in PCG eyes compared to unaffected eyes (all P<0.05). TIA and ciliary process length in unaffected eyes were smaller than PCG eyes