Abstract: Primary cardiac tumors are rare, and clinical epidemiological statistics of the circulatory system is the lowest compared with other systems. Due to the particularity of age, primary cardiac tumors in infants and children are extremely rare, with an estimated incidence of 0.2% according to echocardiographic studies and the autopsy rate in the population is lower. Primary cardiac tumors include benign and malignant:according to statistics, about 90% of primary cardiac tumors are benign, with myxoma the most common and more common with rhabdomyoma; whereas malignant tumors are common with sarcomas. Its incidence is low, but with the progress of clinical auxiliary examination, the prenatal diagnosis rate increases, detection rate of heart damage also increased year by year. Between 1995 and 2005, echocardiography was the most widely used instrument as a non-invasive test. And 2005 CT began to be used in clinical application, while MRI in 2010 can be more effectively detected and assessed of the prognosis and efficacy of cardiac tumors. Occurring in children with heart tumors are mostly primary or no obvious clinical symptoms, but surgery is required only in a minority of cases. Patients with primary cardiac tumors often show as nonspecific symptoms of dyspnea, chest pain, heart palpitations, fever, and weight loss and so on; severe cases can also cause heart rupture, heart and kidney failure, neurological symptoms. Most heart lumps are not suitable for percutaneous biopsy, but pathological findings after surgical resection can prompt a clear diagnosis. At present, the clinical diagnosis mainly depends on echocardiography (transthoracic or transesophageal echocardiography) and cardiac magnetic resonance (MR), and there are some limitations in the rapid and accurate diagnosis of cardiac tumors, which, to some extent also limits the diagnosis of treatment programs, and timely and effective treatment of prognosis is particularly important. However, clinical diagnosis and treatment is still more difficult and timely and effective treatment of prognosis is particularly important. Because of its low incidence, the current reports of the disease are mostly case reports. This article focuses on the classification, diagnosis and treatment of primary cardiac tumors.