Objectives <p>The ectodermal origin of enamel and skin suggests a potential link between dermatological conditions and dental anomalies. This study aimed to evaluate the prevalence of developmental defects of enamel (DDE) and other dental anomalies in pediatric patients with skin diseases.</p> Materials and methods <p>This cross-sectional study included 71 pediatric patients (aged 4–14) diagnosed with chronic skin diseases or genodermatoses, along with 41 age- and sex-matched controls. Enamel defects were assessed using both the Modified DDE Index and the Molar-Incisor Hypomineralization (MIH) Index, while panoramic radiographs were analyzed for hypodontia, taurodontism, short root anomaly, and primary failure of eruption.</p> Results <p>DDE-not-related MIH was significantly higher in the study group (<i>p</i> = 0.01). Additionally, other dental anomalies were significantly more prevalent in the study group (<i>p</i> = 0.03), particularly among patients with genodermatoses (<i>p</i> &lt; 0.001). Ectodermal dysplasia and epidermolysis bullosa exhibited the highest prevalence of dental anomalies.</p> Conclusion <p>Our findings suggest a possible association between skin diseases, particularly genodermatoses, and an increased prevalence of dental anomalies. These results highlight the importance of early dental screening and multidisciplinary care for children with dermatological conditions.</p> Clinical relevance <p>Understanding the dental implications of skin diseases can aid clinicians in early diagnosis and preventive strategies, improving oral health outcomes for affected children.</p>