This paper reviews the data in the published literature (PubMed from 1937 to 2011) concerning the medical and surgical management of pediatric limbal dermoids. Current standard medical treatment for grade I pediatric limbal dermoids (ie, with superficial corneal involvment) is initially conservative. In stages II (ie, affecting the full thickness of the cornea with/without endothelial involvement) and III (ie, involvement of entire cornea and anterior chamber), a combination of excision, lamellar keratoplasty, and amniotic membrane and limbal stem cell tranplantation are advocated. Combinations of these approaches seem to yield better and more stable long-term ocular surface cosmesis and fewer complications in comparison with traditional methods of excision and lamellar keratoplasty. Management of amblyopia (i.e. occlusion treatment, chemical penalization with/without spectacle wear, etc) must continue after surgical excision to yield optimal results when or if the surgery is done at a younger age.