Background Orbital pseudolymphoma is one of the rare diseases affecting the ocular adnexa and causing various symptoms requiring histopathological evidence for diagnosis and appropriate treatment with oral steroids or immunosuppressive agents. However, there is less literature available on this disease, thus adding to it will help in the early diagnosis and treatment of the patients. Case presentation A 48-year-old female from a village in South India came to our tertiary care centre with the only complaints of right eye protrusion for 1 year. With a working diagnosis of intraorbital mass, radiological investigations like contrast-enhanced CT and MRI were taken. It showed well-capsulated mass involving the right inferior rectus extending into the intraconal space just abutting the optic nerve. Trans-antral endoscopic biopsy under general anaesthesia was taken and reported as pseudolymphoma proven with CD 3 and CD 20 immunohistochemistry markers. The patient was treated with oral corticosteroids and tapered over a period of 3 months. During the treatment period, the patient developed no diminution of vision or new complaints. Conclusion Orbital pseudolymphomas are benign lymphoproliferative diseases affecting the ocular adnexal tissue. Patients can present with painless ptosis, proptosis, diplopia, or eyelid swelling. Prompt diagnosis and treatment result in the remission of the disease.
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