Giant Chiari network mimics intracardiac tumor in a case of neurofibramatosis

Neurocutaneos syndromes are associated with cardiac or heart related extracardiac tumors, as well as atrial or ventricular septal defect, pulmonary stenosis, coarctation of aorta. Here we describe a case of neurofibromatosis with valvular pulmonary stenosis and giant Chiari network, which mimics a right atrial tumor originating from interatrial septum.