The aim of this report is to describe four cases of non-autoimmune dia
betes that presented in infancy. Three had transient neonatal diabetes
mellitus (TNDM) with diabetic ketoacidosis at onset, followed by comp
lete remission after several months of insulin treatment. While the fo
urth case was initially diagnosed as TNDM, she had renal, hepatic and
pancreatic dysplasia. These cases illustrate that diabetes in infants
can be difficult to diagnose and that patients with TNDM can have a re
currence of diabetes several years later.