A case of hypothalamic sarcoidosis with autopsy findings is recorded. This case and 18 others from the literature with evidence of a sarcoid lesion in the hypothalamus at autopsy are analysed. The symptoms and signs attributed to secondary disturbance of the endocrine system are much less striking and less diagnostic than those attributed to involvement of other centres in the hypothalamus. In the case recorded, the hyper-somnolence could be attributed to hypothermia. The gross striae was a striking feature and their distribution differed from those seen in Cushing’s syndrome. The electrolyte disturbances were varied and the occurrence of hyponatraemia or hypochloraemia has not been previously reported.