Introduction. Inflammatory pseudotumor is an uncommon entity, and its cardiac origin is exceedingly rare. Case History. A previously healthy 27-year-old man was found to have a systolic murmur during preemployment screening evaluation. A transthoracic echocardiogram revealed a 4 × 2.5 cm mass originating from the right ventricle (RV) outflow tract extending into the aortic root. A computed tomography guided biopsy confirmed an IgG4-related inflammatory pseudotumor. Patient was started on oral prednisone with subsequent reduction in mass size. Conclusion. Cardiac inflammatory pseudotumors are markedly rare tumors that should be considered in the differential of intracardiac tumors which otherwise includes cardiac fibromas, myxomas, and sarcomas.