A 29-year-old man presented with diplopia and decreased strength in the left arm. A magnetic resonance image (MRI) showed an extensive hypothalamic and pituitary gland mass, and hormonal studies showed partial hypopituitarism and mild hyperprolactinemia without diabetes insipidus. Biopsies of the hypothalamic lesion and of a mediastinal lymph node demonstrated noncaseating granulomas, and a Kveim-Siltzbach test was positive. He was successfully treated with a regimen of high-dose intravenous methylprednisolone pulse therapy for eight weeks along with a low dose of oral corticosteroids which was maintained indefinitely. An MRI obtained immediately after pulse therapy revealed a substantial reduction in the hypothalamic-pituitary mass, which was maintained in an MRI performed 3 years later. However, hormonal deficits persisted and indefinite hormonal substitutive therapy was required.