Abstract Background Involvement of lacrimal apparatus by Non Hodgkin lymphoma as a first manifestation is extremely rare and only three cases have been reported in children. The purpose of this report is to determine the clinical characteristic of patient with primary involvement of lacrimal apparatus with lymphoma. Case presentation A 7-year-old girl with history of tearing, gradually progressive periorbital redness and painless swelling over right lacrimal apparatus for almost 40 days was referred for treatment in our clinic. Orbital computed tomography revealed lacrimal apparatus mass. Histopathological examination demonstrated B cell type NHL. Following three cycles of cyclophosphamide, doxorubicin hydrochloride, vincristine and prednisone (CHOP regimen) and radiotherapy, the patient was discharged with good condition. Conclusion Although, the primary lacrimal apparatus lymphoma is extremely rare in children but malignant lymphoma should be taken under consideration in acute dacriocystitis.

Keywords: Lymphoma, Non-Hodgkin, Orbit, Lacrimal Apparatus, Dacryocystitis

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