Primary heart tumors are extremely rare, constituting approximately 0.02% of all malignancies. Inflammatory myofibroblastic tumor (IMT) constitutes <5% of primary heart tumors. Until now, IMT of the heart has been described in 21 infants below 1 year of age. Its etiology remains unknown. IMT usually develops within the right atrial and ventricular endocardium. The main clinical symptoms reported in the affected infants involved increasing respiratory failure, cyanosis, and heart murmurs. Histopathologically, IMT is characterized by the myofibroblast proliferation with inflammatory infiltrates composed of plasmocytes, lymphocytes, and histiocytes. Tumor resection is the treatment of choice in IMT. Such tumor location is associated with the high risk of perioperative failure. Steroid therapy and chemotherapy is reported in the literature as a nonsurgical treatment alternative. Here, we present a review of clinical symptoms, diagnostic and treatment options, based on published case reports of IMT in infants, including our 11-month-old patient with IMT located within the pericardium.