Paediatric blistering diseases encompass a diverse group of dermatological disorders characterized by the formation of fluid-filled lesions on the skin, presenting a unique set of challenges in diagnosis and management in the Paediatric population. These conditions often manifest as bullae, vesicles, or pustules, and their etiology can range from autoimmune processes to genetic mutations, infectious agents, or drug reactions. Pemphigus vulgaris and pemphigoid variants, including bullous pemphigoid and epidermolysis bullosa acquisita, represent autoimmune blistering diseases that can affect children, though they are more commonly associated with adults. Inherited blistering disorders such as epidermolysis bullosa (EB) exhibit a genetic basis and result from mutations in structural proteins crucial for skin integrity, leading to fragile skin prone to blistering. Additionally, dermatitis herpetiformis, a cutaneous manifestation of gluten sensitivity, may present with blistering lesions in Paediatric patients. The clinical evaluation of Paediatric blistering diseases requires a meticulous approach, often involving a combination of clinical history, physical examination, histopathological studies, and immunofluorescence testing. Timely and accurate diagnosis is essential for appropriate intervention and management strategies, which may include systemic immunosuppressive therapies, wound care, and supportive measures to minimize disease-related complications. The psychosocial impact on Paediatric patients and their families should not be underestimated, necessitating a multidisciplinary approach involving dermatologists, Paediatricians, geneticists, and other specialists to address both the medical and emotional aspects of care. Ongoing research efforts aim to deepen our understanding of the pathophysiology of these conditions, paving the way for the development of targeted therapies and improved outcomes for children affected by blistering diseases. Despite the complexity of these disorders, advancements in diagnostic tools and treatment modalities offer hope for enhanced quality of life and long-term well-being for Paediatric patients grappling with these challenging dermatological conditions.