Abstract Purpose: M. Fabry is a rare lysosomal storage disease affecting among other organs the vascular endothelium. Methods: We present a 38 years old male Fabry patient with a prior history of 6 different episodes of stroke. We examined him 3 months after his last stroke in our department for newly developing ocular motility disorders. Results: Slit lamp exam revealed bilateral cornea verticillata, conjunctival vessel anomalies and tortuous retinal vessels. Bilateral blepharospasm pronounced on the right side was present. Bilateral rotatory nystagm was also noted. MRI showed diffuse white matter lesions. For the blepharospasm botolinum therapy was instituted. Conclusions: Most likely Fabry disease causes cerebral ischemia in an atypical pattern, in our patient affecting among others different nuclei responsible for ocular motility.