Background: Congenital colour vision deficiency (CCVD) is an untreatable disorder which has lifelong consequences. Increasing use of colours in schools has raised concern for pupils with CCVD. This case-control study was conducted to compare behavioural and emotional issues among age, gender and class-matched pupils with CCVD and normal colour vision (NCV). Methods: A total of 1732 pupils from 10 primary schools in the Federal Territory of Kuala Lumpur were screened, of which 46 pupils (45 males and 1 female) had CCVD. Mothers of male pupils with CCVD (n=44) and NCV (n=44) who gave consent were recruited to complete a self-administered parent report form, Child Behaviour Checklist for Ages 4-18 (CBCL/ 4-18) used to access behavioural and emotional problems. The CBCL/ 4-18 has three broad groupings: Internalising, Externalising and Total Behaviour Problems. Internalising Problems combines the Withdrawn, Somatic Complaints and Anxiety/ Depression sub constructs, while Externalising Problems combines the Delinquent and Aggressive Behaviour sub constructs. Results: Results from CBCL/ 4-18 showed that all pupils from both groups had scores within the normal range for all constructs. However, results from the statistical analysis for comparison, Mann-Whitney U test, showed that pupils with CCVD scored significantly higher for Externalising Problems (U=697.50, p=0.02) and Total Behaviour Problems (U=647.00, p= 0.01). Significantly higher scores were observed in Withdrawn (U=714.00, p=0.02), Thought Problems (U=438.50, p<0.001) and Aggressive Behaviour (U=738.00, p=0.04). Odds ratios, 95% CI, showed significant relative risk for high Total Behaviour Problem (OR:2.39 ,CI:1.0-5.7), Externalising Problems (OR:2.32, CI:1.0-5.5), Withdrawn (OR:2.67, CI:1.1-6.5), Thought Problems (OR:9.64, CI:3.6-26.1) and Aggressive Behaviour (OR:10.26, CI:3.4-31.0) scores among pupils with CCVD. Conclusion: Higher scores among CCVD pupils indicates that they present more behavioural and emotional problems compared to NCV pupils. Therefore, school vision screenings in Malaysia should also include colour vision to assist in the early clinical management of CCVD children.