胆管内乳头状肿瘤(IPNB)是一种起源于胆道上皮的罕见肿瘤,病因及发病机制尚不清楚。近年来在临床上的发病率逐渐上升,其复杂的病理特征和多样的临床表现给诊断和治疗带来了挑战。本文介绍了IPNB的分类、病因及发病机制、病理学特征及其临床表现,详细叙述了当前国内外关于IPNB的临床诊断方法,包括影像学技术和病理学检查,并评述了这些方法的优势和局限性。同时,也归纳了IPNB目前的治疗原则及方法,分析了治疗效果及预后因素。尽管IPNB的诊断和治疗已取得一定进展,但仍存在诸多挑战,未来需要更深入的研究以优化诊断策略和提高治疗效果。Intraductal papillary neoplasm of the bile duct (IPNB) is a rare tumor originating from the biliary epithelium, with its etiology and pathogenesis remaining unclear. In recent years, the clinical incidence of IPNB has gradually increased, and its complex pathological features and diverse clinical manifestations pose challenges for diagnosis and treatment. This article introduces the classification, etiology and pathogenesis, pathological features, and clinical manifestations of IPNB. It provides a detailed overview of current clinical diagnostic methods for IPNB, both domestically and internationally, including imaging techniques and pathological examinations, and evaluates the advantages and limitations of these methods. Furthermore, it summarizes the current treatment principles and methods for IPNB, and analyzes the treatment outcomes and prognostic factors. Despite some progress in the diagnosis and treatment of IPNB, many challenges still remain. Further in-depth research is needed in the future to optimize diagnostic strategies and improve treatment outcomes.