Sjögren’s syndrome is a rare systemic autoimmune disease in children and can manifest with vague or unusual symptoms, leading to delays in diagnosis. Through ongoing collaboration with the international childhood Sjögren’s syndrome workgroup, the CARRA childhood Sjögren’s syndrome workgroup has collected a cohort of 291 patients with a clinical diagnosis of childhood Sjögren’s syndrome to study its natural history. We propose that the current 2016 ACR/EULAR adult classification criteria may not capture the spectrum of disease manifestations in childhood Sjögren’s syndrome. Using this existing data and examining salivary gland biopsy slides, we will compare disease manifestations in childhood Sjögren’s syndrome that influence the likelihood of obtaining a biopsy, and examine the relationships between salivary gland biopsy findings and disease characteristics. We anticipate that these studies will improve the diagnosis of childhood Sjögren’s syndrome and aid future studies.