Juvenile Sjögren's syndrome is a rare condition that affects children and adolescents with distinctive clinical features. Parotid swelling usually precedes regular oral and ocular symptoms, while typical serological findings may be absent. Hence, diagnosing juvenile Sjögren's syndrome may be challenging to the attending pediatric dentist, and long-term management without proper diagnosis may be jeopardized. The purpose of this report was to detail the features of an adolescent with primary Sjögren's syndrome diagnosed 12 years after the onset of the disease. An updated literature review highlighted the unusual aspects of the pediatric form of the disease.

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