The 2007 World Health Organization (WHO) classification of central nervous system tumours introduces new entities, variants, and histological patterns. The classification is divided into three categories: clinico-pathological entities, variants of entities, and histological patterns. New entities are characterized by distinct morphology, location, age distribution, and biological behaviour. Variants are defined by reliable histological identification, clinical relevance, and association with previously defined entities. Histological patterns are defined by identifiable appearances with no distinct clinical or pathological significance. Eight new entities were included: angiocentric glioma, atypical choroid plexus papilloma, extraventricular neurocytoma, papillary glioneural tumor, rosette-forming glioneural tumor of the 4th ventricle, papillary tumour of the pineal region, pituicytoma, and spindle cell oncocytoma of the adenohypophysis. Angiocentric glioma is a benign tumour of childhood and young adults, often presenting with refractory epilepsy. Atypical choroid plexus papilloma is a Grade II tumour with intermediate features, higher mitotic activity, and increased recurrence. Extraventricular neurocytoma is a rare tumour with similar biological behaviour to central neurocytoma. Papillary glioneuronal tumour is a rare, low-grade tumour. Rosette-forming glioneuronal tumour of the 4th ventricle is a rare, slowly growing tumour. Pituicytoma is a low-grade glioma of the neurohypophysis. Spindle cell oncocytoma of the adenohypophysis is a WHO Grade II neoplasm. Three new variants were included: pilomyxoid astrocytoma, anaplastic medulloblastoma, and medulloblastoma with extensive nodularity. Pilomyxoid astrocytoma is a tumour associated with neurofibromatosis type 1, often located in the hypothalamic region. Medulloblastoma variants are Grade IV neoplasms with distinct imaging features. The 2007 classification reflects updated understanding of tumour biology and imaging characteristics.The 2007 World Health Organization (WHO) classification of central nervous system tumours introduces new entities, variants, and histological patterns. The classification is divided into three categories: clinico-pathological entities, variants of entities, and histological patterns. New entities are characterized by distinct morphology, location, age distribution, and biological behaviour. Variants are defined by reliable histological identification, clinical relevance, and association with previously defined entities. Histological patterns are defined by identifiable appearances with no distinct clinical or pathological significance. Eight new entities were included: angiocentric glioma, atypical choroid plexus papilloma, extraventricular neurocytoma, papillary glioneural tumor, rosette-forming glioneural tumor of the 4th ventricle, papillary tumour of the pineal region, pituicytoma, and spindle cell oncocytoma of the adenohypophysis. Angiocentric glioma is a benign tumour of childhood and young adults, often presenting with refractory epilepsy. Atypical choroid plexus papilloma is a Grade II tumour with intermediate features, higher mitotic activity, and increased recurrence. Extraventricular neurocytoma is a rare tumour with similar biological behaviour to central neurocytoma. Papillary glioneuronal tumour is a rare, low-grade tumour. Rosette-forming glioneuronal tumour of the 4th ventricle is a rare, slowly growing tumour. Pituicytoma is a low-grade glioma of the neurohypophysis. Spindle cell oncocytoma of the adenohypophysis is a WHO Grade II neoplasm. Three new variants were included: pilomyxoid astrocytoma, anaplastic medulloblastoma, and medulloblastoma with extensive nodularity. Pilomyxoid astrocytoma is a tumour associated with neurofibromatosis type 1, often located in the hypothalamic region. Medulloblastoma variants are Grade IV neoplasms with distinct imaging features. The 2007 classification reflects updated understanding of tumour biology and imaging characteristics.