A 24-year-old woman developed headache and rapidly progressive visual distu
rbances during the last trimester of her first pregnancy. Magnetic resonanc
e tomography (MRI) of the brain documented an intra- and suprasellar mass l
esion. For preservation of vision, transsphenoidal microsurgical decompress
ion was performed. Immediately postoperatively, visual acuity improved and
hemianopia resolved. Histological examination yielded the diagnosis of prim
ary lymphocytic adenohypophysitis. This is a rare inflammatory pituitary di
sease. There are no typical clinical, laboratory, or radiological findings
that allow precise preoperative diagnosis. Even though this autoimmune diso
rder is principally steroid-responsive, an improvement of visual disturbanc
es under steroid therapy cannot be predicted, Therefore, surgery is justifi
ed not only to establish the diagnosis, but also to restore vision by decom
pression of the optic nerves and the chiasm.