Objective　To explore the diagnosis and treatment of childhood primary Sjogren syndrome (pSS) with central nervous system symptoms as the first manifestation. Methods　The clinical data of an 11-year-old male with pSS which had onset with fever and headache was retrospectively analyzed. The related literatures were reviewed. Results　The subject was diagnosed with pSS by autoantibody detection, lip biopsy, and ophthalmologic examination. The symptoms were improved after immunosuppressive therapy. According to the literature, the incidence of childhood pSS was low, in which the incidence with involvment of the nervous system as primanry manifestation was even lower, and headache is the most common symptom of central nervous system. The application of corticosteroids and immunosuppressants may help improve the conditions. Conclusions　Involvement of central nervous system in childhood pSS is not typical. Detection of autoantibodies and lip biopsy are helpful for diagnosis.