A 51-year-old man with good health previously presented with a 1-month history of shortness of breath on exertion. Transthoracic echocardiography revealed marked biventricular hypertrophy, mild mitral regurgitation, and moderate tricuspid regurgitation, suggestive of hypertrophic cardiomyopathy (Figure 1A and 1B; Movie I and Movie II). In the parasternal short-axis view, thickening of the left atrial wall and para-aortic tissue were evident, which is unusual in typical hypertrophic cardiomyopathy, a disease that primarily affects the ventricular myocardium ( Figure 1C and Movie III). Computerized tomographic arteriography revealed no coronary artery disease. His heart failure symptoms worsened, and atrial fibrillation developed. At the same time, maculopapular skin rash developed on the upper trunk and the head and neck region. Hypercalcemia with an adjusted serum calcium level greater than 4 mmol/L was recorded. Other blood chemistry and hematologic examinations were unremarkable. His conditions deteriorated rapidly and, despite maximal supportive measures, he died 1 week after hospitalization. Limited clinical postmortem examination revealed a well-built gentleman with a heart weighing 790 g ( Figure 2A). The walls of all 4 chambers of the heart were diffusely thickened by tan-colored infiltrative growth. The left and right ventricular walls were 2.5 and 1.5 cm thick, respectively ( Figure 2B). The cross-sectional area of the superior vena cava was significantly reduced, with nodules resulting from subendothelial infiltrate. The leaflets of valves were not involved. Mild atherosclerosis was seen in the coronary arteries and major vessels. About 50 mL of straw-colored pericardial effusion was also noted. The maculopapular skin rash was distributed over the head and neck region, extending down to the umbilicus. Hemorrhage is seen superficially at the skin lesions. Microscopic examination revealed that diffuse large B-cell lymphoma (DLBCL) had infiltrated the myocardium and replaced a substantial amount of the cardiac muscle bulk ( Figure 2C). The atypical lymphoid cells were large-to medium-sized, with abundant basophilic cytoplasm and large, moderately pleomorphic nuclei ( Figure 2D). Prominent, centrally located nucleoli were also noted. These atypical lymphoid cells were immunoreactive to CD20 and were negative to T-cell markers such as CD3, CD5, and CD10. Epstein-Barr virus-encoding small nuclear RNA was not detected. Frank myocardial infarction was not detected in the residual myocardium, although contractile band necrosis can be seen in individual cardiomyocytes ( Figure 2D, arrows). Infiltration of atypical lymphocytes was also noted in the pericardium. In the skin, perifollicular and perivascular infiltrations of these lymphoma cells were also detected.Cardiac involvement with lymphoma was the most significant clinical and pathological process in our patient. Our case fits into the broader clinical definition of primary cardiac lymphoma. 1 The incidence of cardiac lymphoma in postmortem examination is l...