Purpose of Review <p>Neonatal and syndromic diabetes are rare but important conditions. These conditions often result in severe insulin deficiency or insulin resistance. In this review, we aim to discuss the clinical characteristics and genetics of neonatal and syndromic forms of diabetes.</p> Recent Findings <p>Beyond the development of diabetes mellitus, many other organ systems are affected. Understanding the pathophysiology of these conditions have improved our collective understanding of the genetics and developmental biology related to glucose metabolism and beyond.</p> Summary <p>This review will provide new information for researchers and provide a helpful resource for clinicians when evaluating a patient for neonatal and syndromic forms of diabetes.</p>