RESUMO
INTRODUCTION: Langerhans cell histiocytosis is a multivisceral pathology. Neurological manifestations are rare. EXEGESIS: We report the case of a 31 year old man hospitalized for left partial motor seizure revealing a right frontal tumor with criteria for histiocytosis X. The histological and biological examination found criteria for Langerhans cell histiocytosis (CD1a and S100 reactivity). The check-up for extracerebral localisations of the disease was negative. The outcome was favourable after a total surgical resection. The review of the literature and a discussion on neurological manifestations of this disease were carried out. CONCLUSION: A neurological manifestation can be the first and only symptom of a Langerhans cell histiocytosis.