Tumor necrosis factor alpha (TNFα) blockade has recently been found to be ineffective in treating glandular and extraglandular manifestations of adult Sjögren syndrome (SS), including arthralgia and arthritis. We report a girl who developed purpura, polyarthritis, uveitis, and severe dental caries in the first year of life and optic neuritis by age three. SS was diagnosed at 11 years of age, when severe hypokalemic renal tubular acidosis developed during infliximab treatment for arthritis. In contrast to her other disease manifestations, the arthritis responded remarkably well to TNFα blockade, suggesting that TNFα blockers may have a role in the treatment of arthritis with pediatric SS. © Clinical Rheumatology 2006.