&NA;This report, which includes 12 cases of opticochiasmatic glioma with diencephalic syndrome, is a 22‐year review of such cases at Indiana University Medical Center. These patients were initially seen because of their failure to thrive, and all were without significant subcutaneous fat. All had pneumoencephalography or ventriculography that showed a suprasellar mass displacing the anterior 3rd ventricle. Surgical confirmation of the diagnosis was obtained in every case. Ten patients underwent radiotherapy with subsequent reversal of the diencephalic syndrome.