Background: Rheumatic heart disease (RHD) patients are at high risk of neurological insults even in absence of atrial fibrillation and traditional risk factors for stroke. White matter lesions (WML) on MRI are surrogates for the same. Methods: We conducted a single center prospective study to compare the prevalence of WML among RHD patients with significant isolated mitral valve disease with and without atrial fibrillation (AF). All patients with other traditional risk factors for stroke were excluded. Results: There were 79 consecutive RHD patients with moderate to severe mitral valve disease, 64(81%) with AF. The proportion of WML in patients with sinus rhythm and AF was similar, 20% and 17.2% respectively. Among all, 14(17.7%) patients had WML. There was no significant difference in the age, proportion of gender, type of valvular lesions, rhythm, mitral valve characteristics, LA size or left atrial appendage (LAA) clot amongst patients with or without WML. None of these parameters predicted the occurrence of WML. The majority of the patients had WML in cortical regions (85.7%). Conclusions: RHD patients having significant mitral valve disease, without history of prior stroke and free of traditional risk factors, had commonly detected WML on brain MRI (17.7%). There was no difference in the prevalence of WML in patients with or without AF.