Langerhans cell histiocytosis (LCH) is a relatively rare and unique benign disease characterized by an abnormal proliferation of immature dendritic cells which usually affects children and young adults. Jaws are involved in less than 10% of children with the disease.

To add on to the literature one more case of this rare and unique disease which accounts for less than 1% of all bone tumors and also to discuss its oral manifestation and management.

Case report

Private maxillofacial surgery institute, Kolkata, West Bengal.

A 2 years and 8 months old male child with solitary swelling over the lower left jaw since one and half months. Lesion was fixed to underlying bone and there was an expansion of buccal cortical plate at the body of mandible.

Enucleation of the lesion performed after assessing history, clinical and radiological dertails and also considering patient's age. Postoperative biopsy result came as LCH presenting as a unifocal eosinophil granuloma. Appropriate surgical intervention resulted in a dramatic improvement in patient's clinical condition. At present, patient is disease-free and asymptomatic.

Surgical enucleation or curettage is a preferred treatment option for maxillary and mandibular lesion. Potential for the unifocal disease to become multifocal should not be underestimated as the disease can be unpredictable. We recommend long-term follow-up due to uncertainty of the disease.