A 41-year-old man was referred to Kyushu University Hospital for evaluation of hypothyroidism and hypocortisolemia. Pituitary function test revealed the deficiency of GH(growth hormone), ACTH(adrenocorticotropic hormone), prolactin and TSH(thyroid stimulating hormone). MRI showed empty sella and agenesis of corpus callosum. Clinical diagnosis was hypopituitarism with midline brain anomaly. Septo-optic-pituitary dysplasia (SOPD) is a syndrome characterized by agenesis of septum pellucidum or corpus callosum, optic nerve hypoplasia and congenital hypothalamic-pituitary insufficiency. Our case had no ocular anomalies, but today it is regarded as a variant form of SOPD. Evaluation of the integrity of midline brain structures in patients with congenital hypopituitarism is thus thought to be important for their etiology.