Introduction. Sjogren Syndrome (SS) is a chronic and progressive autoimmune disease that primarily involves immune-mediated damage to the lacrimal and salivary glands [Carsons S. 2022]. The prevalence varies widely from 0.3-3.0%. Predominantly affects females (female/male ratio 9:1) in their fourth and fifth decades of life [Parisis D. 2020]. Lacrimal gland involvement occurs in form of dry eye in the majority of SS, while dacryoadenitis is an atypical and rare manifestation [Roszkowska A. 2021]. Usually, dacryoadenitis in SS presents a lacrimal gland swelling with eyelid redness, with bilateral presentation in 57% [Singh S. 2021]. Case report. A 45-year-old female developed acute left dacryoadenitis with poor response to local and general antibiotics for 3 months. To establish the etiology, the system workup of the patient included: ophthalmological, ORL, and rheumatological examination, laboratory tests, chest X-ray, contrast cerebral CT, and cerebral MRI. At ophthalmological examination: visual acuity OD/OS= 1.0/1.0, OS erythema, and tenderness of superior eyelid. Enlargement of the lacrimal gland causing an S-shaped margin and the lateral impossibility of opening the palpebral slit, conjunctival chemosis. Schirmer test: OD/ OS= 27 mm/22 mm. Laboratory findings as positive antinuclear antibodies, raised levels of rheumatoid factor, circulating immune complexes, autoantibodies to Ro (SS-A) and La (SS-B)suggestive results in SS; others autoimmunity tests showed normal results (antiphospholipid antibodies, complement components C3 and C4). Imaging tests: enlargement of the left lacrimal gland, both portions (without extraocular muscles involvement). Conclusions. Dacryoadenitis is an atypical manifestation in SS and need an interdisciplinary collaboration with a broad spectrum of laboratory tests, which is essential to establish the etiology of non-infection dacryoadenitis. To control disease progression and inflammatory activity is required systemic corticosteroid therapy and immunosuppressive drugs.