Recurrent parotid gland enlargement is a common disorder in children, while that of auto-immune aetiology is rare. Three children with recurrent parotid swelling had autoantibodies including antinuclear antibody, anti-SS-A (Sjögren syndrome-A), SS-B (Sjögren syndrome-B) antibodies and rheumatoid factor, abnormal sialograms and lymphocytic infiltration of salivary glands, which were consistent with Sjögren syndrome. Initially, all three lacked symptoms of keratoconjunctivitis sicca. During follow up, two patients developed xerostomia and were diagnosed as having primary Sjögren syndrome. Recurrent parotid enlargement appears to be important as an initial manifestation of Sjögren syndrome in children.