Introduction Central diabetes insipidus (CDI) or neurohypophyseal diabetes insipidus is a rare disorder in children caused by an inadequate release of the antidiuretic hormone, arginine vasopressin (AVP). CDI is clinically caracterised by polyuria and polydipsia.

Methods and materials Clinical, auxological, biological and neuroradiological characteristics of 10 children with central diabetes insipidus (CDI) were retrospectively analysed during a period of 18 years.

Results Five girls and five boys with permanent diabetes insipidus were enrolled in the study. The mean age at diagnosis was 6 years five months (range: 4 months -14 years). The major cumulative and often subtle presenting manifestations were: polyuria (n = 10), polydipsia (n = 10), fatigue (n = 2), growth retardation (n = 4), headache (n = 1). All patients had low urine osmolality and elevated plasma osmolality on diagnosis.

The aetiology of central DI was organic in 8 patients, craniopharyngioma in 3 patients, Langerhans cell histiocytosis in 2 patients, neurosarcoidosis in one case, Pituitary stalk interruption in one patient and wolfram syndrome in one patient.

Three patients had anterior pituitary hormone deficiency and growth hormone deficiency.

Polyuria and polydipsia have regressed in all patients put under desmopressin. Patients with craniopharyngioma were operated with a tumour recurrence in one case.

Conclusion DIC is a rare disease in Paediatrics. The circumstances of discovery and positive diagnosis are often easy. The underlying aetiology of CDI in children may not initially be obvious. Long-term surveillance is therefore necessary, especially for the early detection of evolving treatable intracranial lesions.