Sjögren Syndrome (SS), a slowly Progressive disease that has unified, validated diagnostic criteria, with excellent evidence and performance in adults, but not in adolescents. We report a 17 year old teenager with a family history of SS in his sister and mother. He presented with fever and fatigue. He had an elevated C reactive protein, leukopenia, positive antinuclear and anti-Rho antibodies and rheumatoid factor. A scintigraphy showed a severe salivary gland dysfunction. The syndrome in this patient had a pediatric clinical behavior despite his proximity to adulthood.