The purpose of this review was to evaluate the association between developmental enamel defects and children born preterm. An identical search was performed in PubMed and Embase and was limited to human studies and studies written in English, German, Danish, Swedish, or Norwegian. Reviews, case studies, and case series were excluded. A total of 283 articles were identified. Twenty‐three publications, of which 19 were follow‐up studies, two were case–control studies, and two were cross‐sectional surveys, were enrolled in the review. The majority of the studies (n = 17) dealt with enamel hypoplasia of the primary teeth. Thirteen studies reported an association between preterm birth and enamel hypoplasia, and, in addition, few studies reported an increased risk of enamel opacities in the primary teeth, in children with a birth weight <1500 g. Seven studies dealt with enamel disturbances of the permanent teeth, four of which suggested an increased risk of enamel opacities. This systematic review suggests an increased risk of enamel hypoplasia in primary teeth of children born preterm and enamel opacities in very‐low birth‐weight children. A larger number of well‐designed studies are, however, needed in order to increase the validity of the studies.