Achiasmia is a rarely diagnosed visual pathway maldevelopment where all or the majority of nasal retinal fibres fail to decussate at the optic chiasm. It has been identified by neuroimaging and also by visual evoked potential (VEP) asymmetry. VEP asymmetry has not been defined consistently in previous studies. The aim was to study VEP asymmetry to flash stimulation in two children with maldevelopment of the optic chiasm in comparison to control children. Both children had congenital nystagmus, optic nerve hypoplasia with a bilateral small double ring, bitemporal visual field defect and normal colour vision. In child 1 visual acuity in both eyes was 0.1, in child 2 it was 0.2. MRI showed reduced chiasmal size in child 1, while in child 2 it was combined with other midline abnormalities. VEP to monocular flash stimulation showed in both children distinctive occipital distribution, which was not observed in control children. The N2 wave was distributed asymmetrically over the ipsilateral hemisphere to the stimulated eye, while the P2 wave was distributed over both hemispheres. The P2 wave was however better defined over the ipsilateral hemisphere. Flash VEP occipital distribution remained similar in child 1, who was followed from 10 months to 9 years. These cases of achiasmia demonstrate a distinctive VEP asymmetry in the distribution of the flash VEP N2 wave, as well as the expected structural defect determined by neuroimaging.