Introduction: Langerhans cell histiocytosis (LCH) is a rare disorder with variable clinical presentations. Among the endocrine compromise, the LCH has a predilection for the hypothalamic-pituitary axis. However, primary thyroid involvement is rare and usually related to multisystemic disease.

Case Report: We present a 30-year-old woman who requested a medical consultation because of polyuria and was found to have central diabetes insipidus (CDI). The patient was diagnosed 6 months later with hypopituitarism. Pituitary stalk thickening was detected on magnetic resonance imaging (MRI). Hepatomegaly was also found. Liver biopsy revealed nonalcoholic steatohepatitis with cirrhotic evolution. Three years later she was diagnosed with type 2 diabetes and morbid obesity. Shortly after she was referred to the hospital because of lost of recent memory and dyspnea. Laboratory tests were within the normal range. Magnetic resonance imaging revealed a 15×13×22 mm mass involving pituitary stalk and hypothalamic region. On computed tomography scan a diffuse goiter, a thymic tumor and diffuse interstitial lung disease were observed. Transbronchial biopsy was diagnosed of LCH. Positron emission tomography imaging revealed a hypermetabolic pituitary lesion and moderate tracer uptake in the thymo, thyroid and lungs. The patient was given chemotherapy with cladribine and prednisone with excelent tolerance.

Conclusions: In patients with CDI and pituitary stalk thickening, LCH should be considered in the differential diagnosis. Close surveillance for this patient population may be warranted to detect other pituitary hormone deficiencies and even a multisystem disease involving other endocrine glands, such as the thyroid.