Background <p>Juvenile idiopathic arthritis-associated uveitis (JIAU) frequently follows a chronic and complicated course. Janus kinase inhibitors (JAKi) represent a promising new therapeutic option.</p> Methods <p>Retrospective monocentric analysis of 20 children with chronic anterior JIAU treated with tofacitinib (<i>n</i> = 18), baricitinib (<i>n</i> = 1) or upadacitinib (<i>n</i> = 1). Outcome measures: uveitis or arthritis inactivity at any time during follow-up, recurrences after achieving inactivity, sparing of medication, occurrence of new ocular complications, resolution of preexisting macular edema.</p> Results <p>All patients had received methotrexate prior to baseline, and at least two biologics had been ineffective in 18/20 patients. Whilst receiving JAKi medication, uveitis was inactive in eight patients (anterior chamber cell count &lt; 0.5+) at any time during follow-up (mean 9.2 ± 3.3 months), but uveitis relapsed in 6 of them subsequently. In 7 patients, treatment was terminated after ≤ 1 year due to inadequate response of ocular inflammation. During follow-up, new uveitis-related complications occurred in 7 patients (9 eyes), the most frequent were macular edema (8 eyes of 7 patients), and ocular hypertension (5 eyes of 3 patients). Reduction of concomitant systemic medication was possible in 3/14 patients. Any reduction of topical steroids during follow-up was possible in 24/30 eyes, and 23/30 eyes received a lower dose at the last follow-up than they did at baseline.</p> Conclusions <p>Our data show limited effectiveness of JAKi treatment in patients with chronic DMARD-refractory JIAU. The number of patients in whom uveitis quiescence could be observed was low, as was the possibility for reduction of concomitant medication, and the relapse rate was high.</p> Clinical trial number <p>Not applicable.</p>