| Summary: | Abstract Background Klinefelter syndrome is considered one of the most common sex chromosome disorders affecting males. The presence of an extra X chromosome can alter the tendency to develop various cancers, including lymphomas. Lacrimal gland lymphoma is a disease of the elderly, with a median age of presentation of 70 years. Case presentation In this article, we report a case of a 35-year-old Arabian Saudi male with a known case of Klinefelter syndrome who presented to oculoplastic clinic complaining of progressively growing superior-temporal mass in the left eye. After evaluation and imaging, an incisional biopsy from the lacrimal gland mass was obtained and histopathological evaluation showed atypical lymphoproliferative infiltrate consistent with extranodal marginal zone lymphoma of mucosa associated lymphoid tissue. The mainstay treatment was external beam radiotherapy, which showed significant improvement in the case. Conclusion This is considered the first reported case of lacrimal gland lymphoma in young patient with Klinefelter syndrome, which increases the association between Klinefelter syndrome and lymphomas.
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