ABSTRACT Vogt-Koyanagi-Harada (VKH) disease is a rare and serious ocular adverse reaction following COVID-19 vaccination. This study aimed to evaluate the clinical features, diagnosis and management of COVID-19 vaccine-associated VKH disease. Case reports of VKH disease after COVID-19 vaccination were collected up to February 11, 2023 for retrospective analysis. Twenty-one patients (9 males and 12 females) were included, with a median age of 45 years (range 19–78), from three main regions, Asia (12/21), the Mediterranean region (4/21), and South America (5/21). Fourteen patients developed symptoms after the first dose of the vaccine, and 8 after the second dose. Vaccines included mRNA vaccine (10 cases), virus vector vaccine (6 cases), and inactivated vaccine (5 cases). The average time interval from vaccination to onset of symptoms was 7.5 days (range 12 hours to 4 weeks). All 21 patients experienced visual impairment after vaccination, with 20 cases involving both eyes. Sixteen patients showed symptoms of meningitis. Serous retinal detachment was observed in 16 patients, choroidal thickening was observed in 14, aqueous cell in 9, and subretinal fluid in 6. CSF pleocytosis was detected in 7 patients and skin symptoms were found in 3 patients. All patients received corticosteroid therapy, and 8 also received immunosuppressive agents. All patients recovered well, with a mean recovery time of 2 months. Early diagnosis and early treatment are crucial to the prognosis of patients with VKH after vaccination with COVID-19 vaccine. The risk of vaccination against COVID-19 in patients with a history of VKH disease should be evaluated clinically.