AIM: To investigate the ocular development of patients who had unilateral congenital cataract (CC) combined with persistent fetal vasculature (PFV). METHODS: This cross-sectional, observational study included patients who had unilateral CC and PFV and those with isolated unilateral CC. Axial length (AL), keratometry, anterior chamber depth (ACD), lens thickness, and vitreous length were obtained. The ocular biometric parameters of the affected eyes of patients with CC and PFV were compared with the fellow eyes and with the affected eyes of patients with isolated CC. RESULTS: A total of 110 patients were included and divided into 4 groups: group 1 (18 patients with CC and PFV, <24mo), group 2 (22 patients with CC and PFV, ≥24mo), group 3 (35 patients with CC, <24mo), and group 4 (35 patients with CC, ≥24mo). The ALs of the affected eyes were shorter than those of the fellow eyes in group 1 (20.02±1.06 vs 20.66±0.63 mm, P=0.025). While the ALs of the affected eyes were longer than those of the fellow eyes in group 2 (23.18±2.00 vs 22.31±1.06 mm, P=0.044) and group 4 (22.64±1.80 vs 22.02±1.01 mm, P=0.033). The keratometries of the affected eyes were steeper than those of the fellow eyes in group 2 (44.78±1.66 vs 43.83±1.38 D, P=0.041) and group 4 (43.76±1.91 vs 43.34±1.46 D, P=0.043). No difference of ACDs between two eyes was found in all groups (all P>0.05). CONCLUSION: Compared with the fellow eyes, the ALs of the eyes with unilateral CC and PFV are shorter in patients younger than 24mo and longer in those older than 24mo; the keratometries of the eyes with unilateral CC and PFV are steeper in patients older than 24mo and similar with those younger than 24mo. These findings provide further understanding of ocular development in patients with both CC and PFV.