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Purpose: Retinoblastoma (RB) is an intraocular malignancy that often occurs in childhood. Circular RNAs (circRNAs) play crucial roles in regulating the malignant phenotypes of various tumors. This study aimed to explore the role and potential mechanism of circ_0000527 in RB.Methods: The levels of circ_0000527, microRNA-98-5p (miR-98-5p) and X-linked inhibitor of apoptosis (XIAP) were determined by qRT-PCR or western blot. Cell proliferation was detected by 3-(4,5-dimethyl-2-thiazolyl)-2,5-diphenyl-2-H-tetrazolium bromide (MTT) and colony formation assays. Cell apoptosis, migration and invasion were evaluated by flow cytometry, transwell and scratch assays. Xenograft assay was conducted to analyze tumor growth in vivo. The binding relationship between miR-98-5p and circ_0000527 or XIAP was verified by dual-luciferase reporter and RNA immunoprecipitation (RIP) assays.Results: Circ_0000527 and XIAP levels were increased, while miR-98-5p level was reduced in RB tissues and cells. Silencing of circ_0000527 suppressed the proliferation, migration and invasion of RB cells and promoted apoptosis. In addition, knockdown of circ_0000527 inhibited tumor growth in xenograft mice. Besides, circ_0000527 sponged miR-98-5p to regulate RB cell progression, and miR-98-5p targeted XIAP to mediate RB cell development. Moreover, circ_0000527 modulated XIAP expression via sequestering miR-98-5p.Conclusion: Circ_0000527 facilitated RB progression via regulating miR-98-5p/XIAP axis, which provided a promising therapeutic target for RB.