This study evaluated pituitary imaging findings in 13 patients with Langerhans cell histiocytosis (LCH) with diabetes insipidus. Nine patients were evaluated with pituitary magnetic resonance imaging (MRI), 3 with brain computed tomography, and 1 with brain MRI. The infundibulum was thickened in 11 (84.6%) patients, thread-like in 1 (7.7%), and normal in 1 (7.7%). Posterior pituitary intensity was absent in 10 patients (76.9%); in 4 patients, the pituitary gland was small in size, and 2 patients had atrophic pituitary. Three had a small sella. Infundibular thickening and absence of posterior pituitary intensity were the most common radiological findings. MRI imaging should be used to follow up patients with pituitary histiocytosis, and patients with LCH and diabetes insipidus should be followed for pituitary atrophy.