To describe and report a challenging case of primary intraorbital B cells lymphoma and to provide an update review of
the literature, highlighting the importance of the clinical and multimodality imaging assessment and finding. A 52-year-old
Indian male patient, known to have hypertension and type II diabetes presented to Emergency Department complaining of
4 days history of gradual onset blurring of vision, left eye pain and double vision. Clinical examination showed left eye
proptosis. MRI Head and Orbit showed left infraorbital extraconal mass lesion along the inferior aspect of the left orbit;
Positron emission tomography (PET CT scan) whole body showed intensive uptake left orbital cavity and Left testis/
epididymis uptake also compatible with suspected lymphoma activity. Under general anesthesia Incisional biopsy was done.
Histopathological examination showed Diffuse large B-cell lymphoma, NOS (Not Otherwise Specified). Patient received
total two cycles of Hyper-CVAD (cyclophosphamide, vincristine sulfate, doxorubicin hydrochloride and dexamethasone) in
addition of two cycles of R-CHOP (rituximab cyclophosphamide, doxorubicin hydrochloride, vincristine, and prednisolone)
showing regression of the lesion on PET CT scan as well as regression of the symptoms. Orbital lymphoma can be easily
mistaken for another ocular disease due to the slowly progressing nonspecific complaints of the patients. If unspecific orbital
symptoms are present, adequate imaging studies followed by early surgical biopsy will contribute to the early diagnosis. We
should always be suspicious of this diagnosis especially in patients over sixty years of age with slowly growing mass in the
orbit or proptosis non-reacting to specific treatment.