Eosinophilic granuloma is one of the histiocytosis X. It may occur in any bone and tissues, but the one originated from the sternum was rarely reported. Recently, we experienced an eosinophilic granuloma at the manubrium sterni, associated with diabetes insipidus, which was surgically resected. Although intranasal spray of DDAVP has been used for the control of diabetes insipidus, the bony lesion was remitted. With the brief review of the literatures, we report the case.