Background: Langerhans cell histiocytosis (LCH) is a great mimicker. It is a rare hematological disorder, incompletely characterised and understood, often affecting infants and young children. This disease has a wide spectrum of clinical presentations, ranging from an isolated disease with spontaneous resolution to life-threatening multisystem disease. Isolated oral presentation is rare and often occurs early in the disease process and usually gets overlooked. Some of the common oral presentations include gingivitis, periodontitis and tooth rotation. Case report: We report a case of a 1 year old male child who presented with a persistent white patch over the soft palate extending to the hard palate, initially treated as oral thrush and diphtheria by the primary paediatricians and subsequently was diagnosed with multisystem LCH (with liver as risk organ) at our centre . Conclusion: This disease requires a very high index of clinical suspicion, timely diagnosis and treatment for a favourable outcome.