Notochordal tumours consist of a distinct group of neoplasms arising in the axial skeleton having notochordal differentiation, which are unified by the co-expression of cytokeratin and brachyury. Brachyury is the diagnostic hallmark and is the single most important immunohistochemical marker for the diagnosis of a notochordal tumour. The recent WHO classification divides notochordal tumours into four main categories, three of which are malignant, namely, conventional chordoma, dedifferentiated chordoma, and poorly differentiated chordoma. The fourth category consists of benign notochordal cell tumour, which is a benign neoplasm. There are defined radiological and histological criteria to differentiate a BNCT from a chordoma, and they also differ considerably in management and clinical outcome. BNCT is a benign tumour with limited intra-osseous growth, whereas chordomas are slow-growing malignant lesions with progressive destructive growth and capacity for metastasis. Chordomas are treated with surgical excision, and a negative surgical margin is the single most important predictor for recurrence and survival. On the other hand, patients with BNCT are followed up with serial imaging surveillance. There is growing evidence that BNCT has potential for malignant transformation into chordoma, and therefore, there is consensus that once identified, these lesions should be carefully followed up over a long period of time to detect any early transformation into chordomas.