Monocytoid B-cell lymphoma is uncommon, low grade lymphoma originating from monocytoid B lymphocytes. Monocytoid B-cell lymphoma usually presents as a localized lymphadenopathy. Peripheral lymph nodes are most often involved, particularly those in the frequent in the head and neck area. A distinctive feature is the association of monocytoid B-cell lymphoma with autoimmune diseases. Sjogren Syndrome had been present in 22% of patient with monocytoid lymphoma. Extranodal involvement by monocytoid lymphoma was reported in the salivary gland, breast, thyroid, and stomach. There were also occasional extensions to the liver and retroperitoneum. The bone marrow and peripheral blood involvement by monocytoid lymphoma is very rare, which is frequently seen in hairly cell leukemia. Fever, weight loss, and other constitutional signs are usually absent. Most patients have no symptoms, and the only sign is enlarged lymph nodes. The clinical course remains indolent; most patients are in complete remission and recurrence with progression to a high-grade lymphoma of large cell type was recorded only in a few cases. Authors experienced a case of monocytoid B-cell lymphoma associated with Sjogren Syndrome mistaken to simple cervical lymphadenitis in a 60-year-old female. We report this case with a review of literatures.