Primary cardiac tumor in infancy is an uncommon condition which is rarely accompanied with congenital heart disease. Although these tumors are generally benign, the complication associated with it, such as arrhythmia, outflow tract obstruction, and heart failure, may result in early mortality, when combined with congenital heart disease. Early surgical treatment may reduce complication risk and increase operative success rate which may improve the patient's long-term prognosis. Herein, we report a case of an eight-month-old Chinese baby boy diagnosed with a calcified fibroma combined with ventricular septal defect, the tumor excision and repair of ventricular septal defect were done at the same time.