Background: Although percutaneous transluminal angioplasty and stenting (PTAS) was an effective and safe alternative treatment for severe intracranial atherosclerosis disease (ICAD), the high rate of restenosis remained a major issue for this endovascular procedure. Recently, the application of drug-coated balloons (DCB) in ICAD was developed to reduce restenosis. This systematic review aimed to evaluate the efficacy and safety of DCB angioplasty for ICAD. Methods: We searched relevant databases for eligible studies enrolling ICAD patients treated with DCB. The event rates of restenosis and periprocedural complications in the follow-up period were pooled with random-/fixed-effect models using Freeman-Tukey double arcsine transformation. Heterogeneity tests and publication bias tests were performed. Results: Two hundred and twenty-four ICAD patients treated with DCB from 9 eligible studies were included. Rate of stenosis in the DCB arm before treatment was ranged from 62% to 90% and reported median follow-up was ranged from 3 to 10.7 months. The pooled incidence of restenosis were 5.7% (95% confidence interval [CI] 2.6% 9.7%; I-2 = 0%, p = 0.516) and 5.9% for periprocedural complications (95% CI: 2.5-10.3%; I-2 = 0%, p = 0.649) in follow-up term. Conclusion: With the limitation of the low quality of the available evidence, angioplasty with DCB appears to be effective and safe in severe ICAD. Further larger randomized trials are needed to provide more definitive evidence and to address the ideal clinical context for their application.