A middle-aged woman presented with insidious onset swelling of bilateral lacrimal and parotid glands. Mikulicz syndrome is a symptom complex caused by a variety of systemic disorders like lymphoma, sarcoidosis, amyloidosis, human immunodeficiency virus infection, tuberculosis, etc. Biopsy from her lacrimal glands revealed mantle cell lymphoma. Although involvement of salivary and lacrimal glands as a part of generalized lymphomatous involvement is not uncommon, Mikulicz syndrome as a presenting manifestation of a lymphoma is very rare.