Abstract Background and Aims Extra-intestinal manifestations [EIMs] are frequent in patients with inflammatory bowel diseases [IBD] and are challenging conditions to manage. Tumour necrosis factor alpha antagonists [anti-TNFα] are recognised as the primary therapeutic option. We aimed to summarise evidence on the efficacy of ustekinumab for the treatment of EIMs. Methods We searched in PubMed, Cochrane Library, and Web of Science, up to October 2020, all interventional and non-interventional studies published in English and assessing ustekinumab efficacy for the treatment of EIMs. Results Nine studies [eight retrospective and one prospective] were included, enrolling a total of 254 patients with IBD and EIM. Ustekinumab showed its effectiveness for arthralgia and psoriatic arthritis in 152 patients through three high-quality studies. Conversely, no efficacy was found in axial spondyloarthritis. Psoriasis, pyoderma gangrenosum, and erythema nodosum were assessed in seven studies including 65 patients and showed high response rate to ustekinumab treatment. Promising results for aphthous stomatitis and uveitis were reported, but data were limited to 20 patients from two studies. Conclusions Ustekinumab showed to be an effective option for the treatment of EIMs, especially for dermatological and rheumatological manifestations. However, more data are needed to confirm the role of ustekinumab in this setting.