报告３７例先天性角膜皮样瘤，其中１３例为Ｇｏｌｄｅｎｈａｒ综合征，占３５．１４％。瘤体４ｍｍ以下者１９例，５～８ｍｍ者１４例，９ｍｍ以上者４例。肿瘤位于角膜颞下方的２７例，占７２．９９％。３３例跨越角膜缘，２例位于角膜内。１３例Ｇｏｌｄｅｎｈａｒ综合征者，有副耳１０例，唇裂２例，颊横裂１例，有副耳者其中３例合并骶椎隐裂。对瘤体小于４ｍｍ或瘤体位于角膜浅层者１５例行瘤体单纯切除，对瘤体大于５ｍｍ或病变累及角膜深层者行瘤体切除加板层角膜移植术，并主张早期手术 Thirty-seven cases of congenital keratomachromatosis were reported, of which 13 cases were Goldenhar syndrome, accounting for 35.14%. 19 cases of tumors less than 4mm, 14 cases of 5 ~ 8mm, 4 cases of more than 9mm. Tumors were located in the corneal infratemporal 27 cases, accounting for 72.99%. 33 cases across the limbus, 2 cases located in the cornea. 13 cases of Goldenhar syndrome, 10 cases of vice ears, cleft lip in 2 cases, buccal and transverse cleft in 1 case, 3 cases of co-ear were combined with the sacral crypt. On the tumor less than 4mm or tumor located in the corneal shallow 15 patients with simple excision of the tumor, the tumor is greater than 5mm or lesion involving the cornea deep tumor resection plus lamellar keratoplasty, and advocate early surgery