Summary Multiple congenital ocular anomalies syndrome consists of several abnormalities mainly localised to the anterior segment of the eye. This condition, comprising iridal or ciliary cysts with or without iridal hypoplasia, an excessively protruding cornea (‘cornea globosa’), miotic pupils, retinal dysplasia, cataract, and other lesions, has been reported in several breeds and is strongly related to the silver coat colour, as a result of a dominant mutant allele at the PMEL17 locus. This report describes the macroscopic, ultrasonographic, histological and magnetic resonance imaging findings of the ocular abnormalities in a Comtois mare with multiple congenital ocular anomalies syndrome.