Gaucher disease often occurs in Ashkenazi Jews but is panethnic.2 Type 1 Gaucher disease is characterized by an absenceof central nervous system involvement, in contrast to types 2 and 3, whichhave primary central nervous system disease. Classic descriptions of thisdisease mention multiple ocular and neurologic associations, such as conjunctivalpterygia, strabismus, and trismus with retroflexion of the neck. Vitreousopacities in this disorder were first noted by Cogan et al3 andGass.4 The incidence of vitreous opacitieswas found to be approximately 3% in a series of 80 consecutive patients withtype 1 Gaucher disease.5 Only those whohave undergone splenectomy have a tendency to form vitreous aggregates. Perhapsthis is due to the occurrence of more circulating glucosylceramide in thesepatients, resulting in manifestations in unusual systemic locations, suchas the eye. Our case is, to our knowledge, both the most severe that has beenreported and the first noting retinal vascular tortuosity. The corkscrew vascularpattern is reminiscent of that in Fabry disease, a related disorder of sphingolipidmetabolism. In our case, there were Gaucher cells in the vitreous cavity,while the vitreous gel contained large amounts of glucosylceramide.