To describe the presentation, histopathological characteristics, and management outcomes for corneal myxoma. This one-armed cohort study evaluated histologically confirmed consecutive cases of corneal myxoma. Data were evaluated on demographics, clinical presentation, management, histopathological and immunohistochemical features, and outcomes; visual acuity and corneal clarity. The study sample was comprised of 10 eyes (10 patients). The median age at presentation was 10.5 years. Five eyes had high intraocular pressure, four eyes had decreased distance visual acuity and one eye became discolored. Surgical management included penetrating keratoplasty (8 eyes), phototherapeutic keratectomy (1 eye), and evisceration because of a blind painful eye (1 eye). Postoperative best-corrected distance vision ranged from 20/20 to 20/60 (1 eye), < 20/60 to 20/200 (2 eyes), < 20/200 to 20/400 (1 eye), < 20/200 to light perception (4 eyes) and no light perception (1 eye). The histopathology of these lesions showed typical subepithelial proliferating spindle-shaped cells of mesenchymal origin within a myxoid stroma rich in glycosaminoglycan. The median duration of follow-up was 5 years. Recurrence was observed in an eye that underwent local excision. Corneal myxoma is a rare lesion that is presumably isolated, secondary, and reactive in nature. Surgically management yields reasonably favorable outcomes.