Sjögren's syndrome (SS) is a chronic inflammatory autoimmune disease affecting mainly the salivary and lacrimal glands characterized by lymphocytic infiltration which results in gland destruction and impairment of functions. SS rarely affects pediatric patients, and there are no clear diagnostic criteria as there are with adult SS. The present case reports an atypical case of SS in a 3-year-old female who was closely followed up with for 5 years. The important role of salivary gland ultrasonography (SGUS) in early diagnosis, the change in clinical picture, as well as fluctuation of serology, are noticed.