We present an unusual case of bilateral dacryoadenitis in a middle-aged patient with adult-onset Still's disease (AOSD). We reviewed relevant clinical studies addressing the association between lacrimal lesions and AOSD. A 50-year-old Chinese woman with a 4 year history of recurrent fever and rashes was admitted to the hospital. She had also developed nodules on both eyelids 10 months before admission. After undergoing resection of the left lacrimal gland, the patient received steroids and immunosuppressive therapy. The patient showed good postoperative recovery during the 20 month follow-up. In this case, the pathological examination conducted after orbital surgery helped clinicians differentiate between dacryoadenitis and other orbital lesions. In a review of the literature, dacryoadenitis occurred after the onset of AOSD, and all cases showed non-granulomatous chronic inflammation by histopathology, which indicated that the lacrimal gland may be an inflammatory target and is affected by systemic inflammation in AOSD.