Although the incidence of primary cardiac tumors is approximately 0.28%, they are encountered in every age group and their clinical presentation usually resembles that of other cardiac or systemic diseases. The clinical symptoms of primary cardiac tumors are usually few until they are at an advanced critical stage making early diagnosis and treatment problematic. However, there is a subgroup of patients who may present with extracardiac systemic, endocrinologic or cutaneous manifestations and familiar inheritance allowing for early detection. This article describes the diagnosis and treatment of a young female who presented with an acute thromboembolic complication of an intracardiac myxoma.