Epiblepharon is a relatively common congenital anomaly and has to be distinguished from entropion, a rare congenital condition. Both cause trichiasis and irritation of the globe. Early surgery prevents severe ocular complications. The very rare congenital entropion of the upper lids, in particular, which is based on a tarsal anomaly known as the tarsal kink, requires primary surgical treatment to prevent keratopathy, corneal ulceration, and blindness in newborns. Epiblepharon can usually be treated adequately with a special suture technique; for congenital entropion, however, a skin-muscle resection is often necessary. Congenital entropion in adults can be treated with good results using a modified lid-split technique. In congenital entropion of the upper lid the tarsal kink has to be resected and the anterior lamella transposed upward and tightened. Postoperative treatment with a therapeutic contact lens accelerates healing of the keratopathy. Good results, both functional and cosmetic, can be achieved with the techniques described in this paper.