良性脊索细胞肿瘤（BNCT）是骨肿瘤中的一种罕见实体，通常出现在中轴骨骼中。尽管这些肿瘤通常是良性的，但由于它们的组织学与更具侵袭性的病变（例如脊索瘤）相似，因此它们的诊断和治疗提出了重大挑战。对 BNCT 的临床行为、诊断细微差别和最佳治疗策略的理解不断发展。椎骨良性脊索细胞肿瘤通常无症状，可通过影像学检查发现，应与脊索瘤区分开来，脊索瘤具有更具侵袭性的临床病程。本报告描述了一名患有腰骶部疼痛的 15 岁女孩，并被诊断为良性脊索细胞肿瘤，该肿瘤影响腰椎 S1 椎骨的大部分，强调了所遇到的诊断挑战、放射学和组织学研究的作用，以及肿瘤良性性质的最终确定。本报告强调了诊断椎骨良性脊索细胞肿瘤所采取的方法以及排除鉴别诊断的重要性。通过探索该病例的复杂性，我们为越来越多的有关 BNCT 的文献做出了贡献，旨在提高对这种罕见骨肿瘤的临床认识和治疗策略。

Benign notochordal cell tumours (BNCTs) represent a rare entity within the spectrum of bone neoplasms, which typically arise in the axial skeleton. Although these tumours are often benign, their diagnosis and management pose significant challenges due to their histological similarity to more aggressive lesions, such as chordomas. Understanding of the clinical behaviour, diagnostic nuances, and optimal management strategies for BNCTs continues to evolve.Benign notochordal cell tumours of the vertebra are usually asymptomatic and identified on imaging and should be distinguished from chordomas, which has a more aggressive clinical course. This report describes a 15-year-old girl with lumbosacral pain and a diagnosis of a benign notochordal cell tumour, which affects a large part of the S1 vertebra in the lumbar spine, highlighting the diagnostic challenges encountered, the role of radiological and histological investigations, and the ultimate determination of the benign nature of the tumour.This report highlights the approach taken for the diagnosis of a benign notochordal cell tumour of the vertebra and the importance of excluding differential diagnoses. By exploring the intricacies of this case, we contribute to the growing body of literature surrounding BNCTs, with the aim of improving clinical awareness and management strategies for this uncommon bone tumour.