AIM: To delineate the different imaging characteristics of uveal schwannoma from melanoma and discuss the optimal treatment strategy for intraocular schwannoma. METHODS: Case series of three patients diagnosed with intraocular schwannoma was collected at Zhongshan Ophthalmic Center, Guangzhou, China from July 2014 to December 2020. All the study patients underwent ultrasonography and magnetic resonance imaging (MRI). The clinical features, therapeutic strategies, and prognoses of all patients were reviewed. RESULTS: Ultrasonography of all three patients (all females, mean age, 39y, age range, 23-54y) showed low to medium reflectivity with a homogeneous internal structure. MRI of all three patients demonstrated isointensity on T1-weighted imaging spin-echo (T1WI SE) images and hypointense on fast spin-echo T2-weighted images (FSE T2WI) images with respect to the brain. Minimally invasive pars plana vitrectomy (PPV) and local resection of the tumor was performed for all patients, and the diagnosis of schwannoma was confirmed by histopathological examination. CONCLUSION: The present study indicates that ultrasonography and MRI features of uveal schwannoma may contribute to the differentiation of uveal schwannoma from melanoma, and the optimal therapy for intraocular schwannoma is minimally invasive PPV and local resection.