A 12-year-old girl presented at the Oncoemato-logic Department with an acute onset of generalized lymphadenopathy. Lymphoproliferative disorders were highly suspected. Biopsied cervical and inguinal lymph node disclosed neither malignant cells nor monoclonal proliferation of lymphocytes. Revaluating the diagnosis, anamnestic data revealed multiple episodes of bilateral parotid swelling since age one, without systemic symptoms. Laboratory investigations, parotid ecography, Schirmer test revealed Sjogren's syndrome without associated disease. Sjogren syndrome (SS) in childhood is a rare and possibly underdiagnosed condition.