We report two cases of mirror image anomalies in two different pairs of monozygotic twins. In case 1, the twins exhibited mirroring of strabismus and refractive errors. Twin 1 had 35 prism diopters (PD) right intermittent exotropia at distant fixation and myopic anisometropia that was spherical 2.00 diopters more myopic in the right eye. Twin 2 had 35 PD left intermittent exotropia at distant fixation and her left eye was more myopic by - spherical 1.00 diopters. In case 2, the twins were diagnosed with infantile nystagmus with upbeat jerk. Twin 1 exhibited a habitual head turn of 30° to the left with dampening of her nystagmus in dextroversion. Twin 2 also exhibited abnormal head position, but in his case the habitual turn was 30° to the right. We believe that this is the first report describing mirror imaged intermittent exotropia with anisometropia and infantile nystagmus with opposite abnormal head positions in pairs of monozygotic twins.