To present the profile of a large cohort of children with persistent fetal vasculature (PFV) and identify their predictors of poor visual outcome in a tertiary eye hospital in Saudi Arabia. This was a single-center study. Medical records of children diagnosed with persistent fetal vasculature between January 1990 and January 2020 at King Khaled Eye Specialist Hospital (KKESH), Riyadh, were reviewed. Data were collected on PFV type, presenting features, management, and visual acuity. Poisson regression with robust error variance was used to identify the predictors of poor visual outcome. Data of 175 eyes of 170 children with PFV were analyzed. Most had unilateral disease (n = 165, 96.2%), and over half of them (57.1%) were males. The main presenting complaints were leukocoria (n = 126, 72.0%), followed by smaller eye (n = 36, 20.6%), poor vision (n = 15, 8.6%), and strabismus (n = 13, 7.4%). Anterior, posterior, and combined PFV were present in 30.9%, 10.9%, and 58.3% eyes, respectively. In eyes with measurable visual acuity (n = 124), BCVA at the last follow-up was ≥20/100, <20/100-20/400, CF/HM and LP/NLP in 11 (8.9%), 32 (25.8%), 43 (34.7%), and 38 (30.6%) eyes, respectively. In the multivariable poisson regression analysis, eyes with posterior PFV were approximately two times more likely to have a BCVA of CF or worse than those with anterior PFV (Adjusted incidence ratio: 1.82 (95%: 1.23, 2.70; P = 0.003). Compared with eyes with primary or secondary IOL implantation, aphakic eyes or those with no cataract surgery were significantly more likely to have BCVA of CF or worse (adjusted incidence ratio: 1.84 [95%: 1.18, 2.86], p = 0.007 and adjusted incidence ratio: 1.64 [95%: 1.08, 2.50], p = 0.020, respectively). The prognosis of PFV varies depending on the severity of the disease. Patients with posterior PFV had worse visual outcome than anterior or combined type. Whether primary or secondary, IOL implantation increases the probability of having BCVA better than CF.