A 75-year-old man presented to our hospital with dry cough, general fatigue, thirst, and polyuria. Four years previously, he had been diagnosed with autoimmune pancreatitis and treated with 30mg/day of prednisolone, which had been tapered to 1mg/day 5 months previously. Chest computed tomography revealed infiltrative shadow in both lungs, and he was admitted. Transbronchial lung biopsy indicated a possible diagnosis of IgG4-related respiratory disease. Head magnetic resonance imaging revealed swelling of the pituitary stalk and loss of high signal intensity in the posterior pituitary on T1-weighted image. Based on the imaging findings and endocrinology examination, we diagnosed the patient as having IgG4-related hypophysitis and central diabetes insipidus. We increased his prednisolone and started desmopressin. Improvement of imaging findings and symptoms was observed.This is a rare case in which IgG4-related respiratory disease and hypophysitis occurred during remission of autoimmune pancreatitis.