A congenital eyelid coloboma is a full-thickness margional eyelid defect which leaves the cornea exposed. Clinical features vary from a simple limited defect with minimal adhesions to the cornea, to complete absence of the eyelid as in cryptophthalmos. Small and moderate-sized upper eyelid colobomas can usually be closed primarily or with simple reconstructive techniques. Larger defects or those associated with corneopalpebral adhesions and craniofacial clefts will require more major reconstructive procedures including grafts and flaps.