Eosinophilic granuloma is the most benign disorder of the triad commonly known as histocytosis X. In this article a case of a 6 year old female child with multiple eosinophilic granuloma with additional liver dysfunction and its oral manifestation is presented. This case demonstrated that oral findings, may be an early manifestation of the disease, definitive diagnosis needs to be determined by correlation of the clinical findings with histologic features. For the duration of 8 years the case has been followed up, there has been a progressive healing of the lesion, the clinical manifestations of the disease resolved with only chemotherapy and provided a very good prognosis.