Rathke's cleft cysts (RCCs) are considered to arise from the remnants
of Rathke's pouch, an invagination of the stomodeum. They are classica
lly described as benign epithelium lined intrasellar cysts containing
mucoid material, and also found in 2-33% of routine autopsy series. Th
e most common presenting symptoms are visual impairment, hypothalamic
dysfunction, hypopituitarism and headache. Diabetes insipidus has been
described in patients with RCC. Very few cases presented with only di
abetes insipidus in adults. To our knowledge, our patient is the first
case of RCC presenting with only diabetes insipidus in childhood. A 9
-year-old girl presented with diabetes insipidus. The physical, neurol
ogical and endocrinological examinations were normal, except for diabe
tes insipidus. Magnetic resonance imaging scan revealed a hyperintense
lesion with supra sellar extension in the posterior pituitary both on
T1 and T2 weighted images. Subtotal excision of RCC was performed via
transsphenoidal surgery. However, diabetes insipidus persisted after
the surgery.