Sjögren's syndrome (SS) is a chronic inflammatory systemic autoimmune disease affecting the exocrine, salivary and lacrimal glands. The condition occurs more often in adults and is rare in childhood. SS should be considered in the differential diagnosis of recurrent parotitis and keratoconjunctivitis sicca. Oral manifestations include early tooth decay and xerostomia. Treatment consists of operative dentistry and saliva management. This paper reports a case of a 10-year-old Brazilian boy with SS, stressing the oral manifestations, treatment conduct, clinical importance and need for an early diagnosis in order to improve the patient's quality of life.