RESUMO
BACKGROUND: The rare form and mildest variant of Langerhans cell histiocytosis is eosinophilic granuloma (EG). In the clinical presentation, EG can be monostotic, polyostotic, or can encompass many organs. The parietal bone is the most common location of the skull bones that are affected by EG. So far, there have been no reported cases of EG with skull odor as an unexplained presentation. CASE PRESENTATION: An 8-year-old girl presented with a 4 months history of a right parietal bone swelling of the skull with an offensive odor. There was no discharge and no history of vomiting or trauma. An MRI scan of the brain showed swelling with a bone lesion of the right parietal bone. Infection was the source of the swelling and the bad odor. Treatment was done by surgical excision of the lesion. CONCLUSION: EG has a variety of presentations and should be suspected when tenderness and local swelling are present. Radiography was found to be helpful in the diagnosis and surgical treatment was done to manage the case.