Kabuki syndrome is a rare genetic disorder, caused by mutation in the KMT2D or 
KDM6A genes, which affects several organs in the majority of patients, among 
which are the eyes. The most typical clinical characteristics are mental 
retardation, postnatal growth retardation, skeletal anomalies, and 
characteristic facial features. As the eyes are affected in most of the cases, 
ophthalmological examination is recommended for the early detection of ocular 
anomalies, in order to prevent visual impairment. The most frequent ocular signs 
are strabismus, ptosis, and refractive anomalies. A series of cases of Kabuki 
syndrome is described in five children, four of whom exhibited strabismus with 
esotropia, over action of inferior oblique muscles, and under action of superior 
oblique muscles associated with a V pattern. Most published papers do not report 
or might underestimate the ocular problems. It may be appropriate to perform 
orbital magnetic resonances in order to detect changes in the muscle paths that 
are related to the pathology of the eye movements found.