We report on a girl with central diabetes insipidus, growth hormone deficiency and bone lesions in multisystem Langerhans cell histiocytosis. Thickening of the pituitary stalk was detected by magnetic resonance imaging, which progressed over the course of the disease. During the observation period she developed primary hypothyroidism, which might be due to the extremely rare involvement of the thyroid gland in this disease. The girl underwent chemotherapy, which led to a regression of the Langerhans cell histiocytosis-lesion, but the hormone deficiencies persisted and substitution had to be continued. Langerhans cell histiocytosis should be included in the differential diagnosis in cases with pituitary stalk thickening and additional hypothalamic/pituitary hormone deficiencies, and in cases of acquired primary hypothyroidism, with or without enlargement of the thyroid gland and ultrasound findings similar to thyroiditis.