The anterior segment dysgeneses are an ill-defined group of ocular developmental abnormalities that share some common features and have a high prevalence of glaucoma. Current classification of what are and what are not anterior segment dysgeneses seems to vary and our knowledge of them is incomplete. As the limits of classical clinical medicine based on evaluation of signs and symptoms are reached, further advancements increasingly will come from molecular medicine and genetics. In this article we review the normal and abnormal development of the anterior segment (concentrating primarily upon neural crest derived dysgeneses), describe the various clinical entities produced and their diagnosis, and discuss the current knowledge of the genetics of these disorders. We also suggest a new approach to the classification of anterior segment dysgeneses, based upon the embryological contribution to the formation of the anterior segment of the eye.