Childhood-onset Sjögren’s disease (cSjD) is a rare and complex autoimmune disease with heterogeneous clinical features ranging from mild glandular symptoms to severe systemic involvement. Previously neglected due to its low prevalence—a diagnosis before 18 years of age is reported in only 1% of all Sjögren’s disease (SjD) cases —cSjD has recently gained attention from both clinicians and researchers as a clinically distinct condition affecting adolescents and young adults (AYA), which deserves early recognition and prompt management because of its potentially negative impact on quality of life (QoL) and risk of serious complications. Compared with adult-onset disease, children and AYA have a high prevalence of recurrent parotitis and systemic or extra-glandular symptoms. Early diagnosis is critical, but the classification criteria used for adults may not be sensitive enough for AYA. In addition, a large percentage of AYA have not undergone all the necessary diagnostic tests according to adult criteria. Therefore, most cases are diagnosed based on expert opinion. A multidisciplinary approach is the cornerstone for managing these AYA with cSjD, addressing both the glandular and extra-glandular components of the disease. Treatment combines topical and systemic therapies to manage symptoms and prevent long-term damage. Regular dental and ophthalmologic follow-up is essential. Vaccination and patient education in self-management and lifestyle changes are critical, along with psychological support. Transitioning to adult care requires careful planning to ensure continuity and comprehensive management. Despite challenges in disease recognition and lack of evidence-based treatments, early and effective management can potentially exert a significant positive impact on the outcomes and QoL of AYAs with cSjD.