Cortical visual impairment (CVI) is now the most common cause of visual impairment in children. Little is known about the long-term visual outcome. This study evaluates the outcome of children with congenital CVI. Using medical records, 423 children (225 males, 198 females) were identified with congenital CVI. Of these children, 259 had follow-up visual acuity assessments. The children's gestational age varied with 32 weeks or less representing 15.9%; 33 to 36 weeks representing 10.7%; 37 to 42 weeks representing 61.2%; and 43 weeks or greater representing 0.9% (11.3% of patients' gestational age was unrecorded). Clinical data were extracted and information regarding outcome was gathered. The majority of children showed improvement in their visual acuity levels after 2 or more years of follow-up. For the 194 children initially assessed before 3 years of age, 97 had improved, 75 were unchanged, 18 had deteriorated, and 4 had sub-optimal assessments. For the 74 children initially assessed at 3 or more years of age, 23 had improved, 44 remained unchanged, 3 had deteriorated, and 4 had sub-optimal testing. Children with better visual acuity levels at follow-up were more likely to have favourable cognitive outcomes (non-mental retardation) in 12.2% versus 2.8% (p<0.01). Similarly, favourable motor outcomes (independent ambulation) were present in 20.1% for those with better visual acuities versus 7.9% for those with poorer visual acuities (p<0.01). Our study demonstrates that the majority of children with CVI underwent improvement in visual acuity. Additional disabilities were common, but those children with better visual acuity outcomes faired better. Given the frequency of comorbid conditions, appropriate diagnostic assessment services are needed.