A 19-year-old male Siberian tiger was presented with inappetence and paralysis of hind limbs. In a computed tomography (CT) scan, intervertebral disk disease at L3–L4 was detected. Cardiac arrest occurred during the surgery. At autopsy, myxomatous mitral valve degeneration (MMVD) and eccentric hypertrophy of the left heart were noted. The diagnosis was congestive heart failure caused by MMVD. Microscopically, myocardial and pulmonary fibrosis were observed in addition to the disintegration of the fibrosa layer and accumulation of glycosaminoglycans and proteoglycans in the spongiosa layer of the mitral valve. This is the first case of congestive heart failure with MMVD in a Siberian tiger.