Infective endocarditis (IE) of the mitral valve (MV) manifesting paravalvular abscess (PA) is challenging. A 30-year-old woman presented with PA fistulating to the left ventricle, detachment of the coronary sinus and systemic embolization. During a course of fever of unknown origin, the patient received laparoscopic surgery under the diagnosis of strangulating intestinal obstruction due to colitis. Following abdominal surgery, abscess having blood flow within it from the left ventricle was pointed out at the left ventriculo-atrial junction by transthoracic echocardiography. Emergency surgery was performed under the diagnosis of PA of MV. Abscess debridement followed by reconstruction of the mitral annulus and coronary sinus with fresh autologous pericardium and mitral valve replacement (MVR) using a mechanical prosthetic valve were successfully performed. Timely and accurate diagnosis followed by the early surgical intervention with aggressive debridement of abscess and reconstruction with autologous pericardium should improve the outcome of this high-risk disease.