Primary corneal myxoma is extremely rare. It has only been reported on 2 previous occasions. Secondary corneal myxomas are more common, arising from corneal diseases such as infective keratitis, keratoconus, and bullous keratopathy. Myxomas occur commonly in other soft tissues such as the heart, paranasal sinuses, and muscles but can rarely present in periocular structures including the conjunctiva, orbit, and eyelid. Ours is only the third case of primary corneal myxoma reported in the literature and illustrates several unusual features. These include an inferonasal location between the corneal epithelium and Bowman layer and with no relationship to the corneal stroma, rapid tumor growth over a 3-month period, and no previous ocular trauma or conjunctival pathology. The histology of this lesion has an important part to play in the management of this condition as it determines the cellular origin, establishes a benign or malignant state, and helps with treatment and prognosis. One reported case of primary corneal myxoma recurred within 2 months after local resection. This was treated with bandage soft contact lens, and no recurrence had been reported since. Our case is now 12 months post op and has had no recurrence.