The clinical diagnosis of rheumatic heart disease (RHD) is challenging. Echocardiography has greatly improved diagnostic sensitivity and specificity and should be used during diagnostic evaluation when available. This chapter outlines the clinical and echocardiographic diagnosis of RHD. It considers individuals with and without a history of acute rheumatic fever (ARF), the latter being the most common scenario globally. We describe the various manifestations of RHD and how to assess and grade the severity of valvular dysfunction while also highlighting knowledge gaps in how we evaluate multivalve and mixed-valve disease. Finally, we discuss cardiac CT and MRI, which can provide supplementary information to guide cardiosurgical management strategies.