The Pathways project was undertaken to devise guidelines to facilitate rapid diagnosis of paediatric brain tumours. Methods: A systematic review and meta-analysis of published data on paediatric brain tumour presentation and analysis of the presentation of children newly diagnosed with a brain tumour at four oncology centres was undertaken. The results informed a professional consensus process. Results: 74 papers met the inclusion criteria for the meta-analysis. 56 symptoms and signs at diagnosis were identified. The most frequent symptoms and signs at diagnosis were: headache (33%), nausea and vomiting (32%), abnormalities of gait and coordination (27%), and papilloedema (13%). 139 patients were recruited to a multi-centre cohort study. Symptoms and signs at disease onset and at diagnosis and factors associated with a long and short symptom interval were determined. A shorter symptom interval was associated with nausea and vomiting and motor system abnormalities. A longer symptom interval was associated with head tilt, cranial nerve palsies, endocrine and growth abnormalities and reduced visual acuity. A multi-disciplinary workshop and Delphi consensus voting were used to translate the evidence into a clinical guideline comprising 76 statements advising on the identification and assessment of children who may have a brain tumour.