Background Fungal disease is one of the most common complications of m
alignant haematologic disease. In the major part of the patients the l
ungs and the gastrointestinal tract are affected. Infections of the ey
es and adnexa are very rare. Patient and methods A 75-year-old woman p
resented with acute unilateral loss of vision. The history revealed an
anaplastic anemia. Vision of the affected eye decreased to light perc
eption. The ophthalmoscopic findings were normal. A retrobulbar tumor
was diagnosed by ultrasonography and CT. It was regarded as a glioma o
r a meningeoma. A Non-Hodgkin lymphoma of low malignancy was proven by
biopsy of bone marrow; hence a retrobulbar lymphoma was included in t
he differential diagnosis. Radiation was started, but the general stat
e of health worsened and the patient died. Autopsy findings A severe m
ycotic infiltration of the left optic nerve sheath was found, which ha
d broadened the spatium intervaginale. Cause of death was a pneumonia
by generalized immunocytoma. Conclusions In cases with retrobulbar tum
ors and malignant haematologic diseases fungal infections should be in
cluded in the differential diagnosis. Serologic investigations are use
ful. The prognosis is poor and depends on the course of the basic dise
ase.