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INTRODUCTION. Paediatric pseudotumour cerebri or idiopathic intracranial hypertension syndrome is rare in the paediatric age and presents with clinical features that differ from the clinical picture seen in adults. It is not a benign condition in children and, although sometimes used to identify this syndrome, the term ‘benign intracranial hypertension’ must therefore be avoided. It is characterised by an increase in the intracranial pressure with analytically normal cerebrospinal fluid and the absence of expansive injuries detected by means of neuroimaging. DEVELOPMENT. It is a complex syndrome that causes intense headaches in children with acute loss of vision and, on occasions, optic atrophy. Its treatment has to be tailored to each patient and the use of different pharmacological or neurosurgical treatments must be considered to avoid permanent visual damage. We reviewed the case mix at our Institute over the last 10 years (1995-2005) and found 23 children (between 3 and 15 years of age) who fulfilled Dandy criteria confirming a diagnosis as suffering from paediatric pseudotumour cerebri, with no predominance of sex or associated obesity.