Introduction:True Primary Cardiac Tumors (PCT) are rare, however they are associated with serious complications. They can be diagnosed and followed by fetal echocardiography (FE) to plan proper management. Objective: To determine accuracy of diagnosis and outcome of PCT diagnosed by FE.Methods: We retrospectively evaluated 1460 consecutive FE performed during March 1990-December 2008 followed by chart review of mothers and newborns with PCT at a tertiary care facility.Results: PCT were diagnosed in 14 Patients [0.001%] referred by obstetrics because of cardiac mass in 12 and 2 with large isolated pericardial effusion (PE). The gestational age at referral was 25-34 weeks.12 patients had rhabdomyoma (RM),9 had Tuberous Sclerosis (TS) including all patients with multiple masses.1 patient with single RM underwent palliation with Blalock-Taussig shunt for right ventricular inflow obstruction with subsequent regression. 1 patient with multiple RM died 12 days postnatally due to ventricular arrhythmias in addition to right ventricular outflow obstruction. The 2 Patients with PE had single right atrial mass; one fetus died in utero due to congenital anomalies and the other underwent successful resection postnatally preceeded by antenatal and postnatal pericardiocentesis.Conclusions:1.Fetal echocardiography is accurate in assessment of PCT 2.PCT should be suspected in fetuses with large isolated PE.3.The most common fetal PCT is multiple RM which is associated with TS.4. PCT can lead to fatal arrhythmias and critical cardiac obstruction.