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Purpose to report visual acuity and strabismus frequency in children with ocular coloboma. Methods We retrospectively analysed 43 children diagnosed as having ocular coloboma (18 boys and 25 girls). Median patient age at diagnosis was 30 months. We studied: visual acuity, binocular status, and refractive errors. We ranked different coloboma as anterior, posterior and antero‐posterior with a whole ophthalmological examination and were divided in two groups: with (I) or without (II) strabismus. Results 43 children were included, 25 (58.1 %) had strabismus, 18 with esotropia (72 %) and 7 with exotropia (28 %). Groups I and II were similar on social issues. 23 children had bilateral coloboma and 20 unilateral coloboma. A total of 66 eyes were studied: we found 5 anterior coloboma, 33 posterior and 28 antero‐posterior. 13 were associated with microphtalmia. There was no significant difference between children in group I and II. 29 Colobomatous anomalies were found in group I and 37 in group II. Children in group I had a worse visual acuity than in group II (p< 0.001). Severe coloboma frequency (posterior or antero‐posterior with macular involution) was higher in group I than in group II. Conclusion These results are comparable to those found in literature. There is a worse visual acuity for strabismus‐associated coloboma.Strabismus more often occurs in coloboma‐diagnosed children than in general population and is often associated with amblyopia.