Abstract
BACKGROUND: Cardiac myxoma with glandular differentiation is a rare finding. It accounts for approximately ≤ 3% of all cardiac myxomas. The presence of cellular atypia resembling adenocarcinoma is an extremely rare finding in cardiac myxoma. CASE PRESENTATION: Here, we present a case of villiform cardiac myxoma with atypical glandular differentiation in a 16-year-old Indo-Aryan girl, which was initially mistaken as metastatic adenocarcinoma on routine histology. CONCLUSION: A thorough histopathological examination, supported by immunohistochemical and radiological correlation, assists in accurate diagnosis and concurrently evades the misdiagnosis of metastatic adenocarcinoma.