Langerhans Cell Histiocytosis of Maxillary sinus

Langerhan cell histiocytosis is a rare disease and usually occurs in paediatric age group. This disease may involve single or multiple organs system and has an unpredictable course of disease. The involvement of head and neck region are almost 90% of cases, however maxillary sinus involvement is very rare. We report a case of 2 year old boy presented with multi organ LCH (orbit, skull, sinus and liver). The mainstay treatment for this high risk multi organ LCH group is chemotherapy. Unfortunately, although with the advancement of treatment, their mortality rate is still high.