Purpose. To report the clinicopathologic findings of a myxoma that arose in the subepithelial region of the right cornea of a 53 year-old man 4 years after successful treatment of an infectious corneal ulcer. Methods. Histopathologic and histochemical evaluation of corneal tissue. Results. This rare lesion appears to have originated from corneal stromal fibroblasts that reacted to an inflammatory stimulus and produced excessive amounts of glycosaminoglycans (hyaluronic acid) rather than normal collagen. Conclusion. Myxoma formation may require interruption of Bowman's layer and proximity of the scar to the epithelium.