OBJECTIVE Congenital abnormalities of the cranium and face present complex diagnostic and therapeutic challenges to the ophthalmologist. RESULTS Patients with craniofacial anomalies are best treated by a multidisciplinary team that includes specialists from plastic surgery, neurosurgery, ophthalmology, otolaryngology, oromaxillofacial surgery, orthodontics, anesthesia, and genetics as well as specialists in support disciplines such as nursing, social work, and nutrition. Other subspecialty needs may be important to address behavioral and developmental issues as well as associated systemic anomalies and medical conditions. The specialists must work together to provide for the overall well being of the patient. To assist in the evaluation and treatment of these patients, the ophthalmologist must possess an understanding of craniofacial syndromes as well as the necessary medical and surgical interventions required to improve ocular and adnexal problems. CONCLUSIONS This article systematically reviews the major craniofacial anomalies of ophthalmic importance and highlights salient treatment issues.