Background: Insulin-dependent diabetes mellitus can be associated to an auto-immune disease. A similar association may be seen in the neonate.
Case report: A one-day-old boy was admitted for ketoacidosis due to diabetes mellitus. Investigation showed that he also suffered from an auto-immune enteropathy with several types of autoantibodies, particularly anti-enterocytes antibodies. Immunotherapy was ineffective. Multi-organ lesions developed subsequently, particularly a pulmonary disease responsible for the death at the age of 2 years.
Conclusion: Physiopathology of this association remains unclear and the report of further cases should help improve our knowledge.