Background: Extremely young children aged ≤6 years old represent a unique population among patients with type 1 diabetes in terms of glycemic variation, diabetes management and complications.Objective: We describe distinct features of diabetes care and outcomes in preschool age children. Methods: We searched PubMed, Google Scholar, and authors’ bibliographies in order to extract articles specific to type 1 diabetes in preschool age children. Findings: The preschool age group is beset by many challenges to diabetes care, including more frequent hypo- and hyperglycemia, hypoglycemia unawareness, decreased residual beta cell function, and greater long-term neurocognitive effects from severe hypoglycemia and chronic hyperglycemia. Randomized controlled trials show that equally good metabolic control can be obtained with multiple daily injections or an insulin pump. Several non-randomized trials, including an 8 year longitudinal study, show lower hemoglobin A1C and decreased hypoglycemia on insulin pumps. Sensor augmented pump therapy resulted in superior A1C as long as sensors were used regularly. In contrast to adults, continuous glucose monitoring has little to no impact on A1C, although parents appreciate the improved monitoring for hypoglycemia. Children with onset of diabetes prior to age 5 are at risk for younger onset of microalbuminuria, however do not develop earlier onset retinopathy than children diagnosed after 5 years. Both severe hypoglycemia and chronic hyperglycemia have negative impact on neurocognition. Conclusion: Special knowledge about this young population is helpful for practitioners and parents.