Papillary fibroelastomas are rare benign heart tumors, with the most common involvement of heart valves. We will present an extremely rare localization of a large Valsalva sinus fibroelastoma, with occasional left coronary artery ostial obstruction, which is presented as an acute coronary syndrome. The tumor was removed surgically and histologically confirmed as papillary fibroelastoma. This review points to the crucial importance of multidisciplinary team decision and multimodality imaging method for diagnosis of the fibroelastoma, determination of size, and localization, which avoided complications of fatal embolizations during an invasive procedure.