Background <p>Cardiac myxomas are the most common primary benign tumors of the heart, with the majority occurring in the left atrium. They are typically composed of gelatinous connective tissue, but rare cases exhibit unusual histological features, including glandular differentiation, which can mimic malignant neoplasms and pose diagnostic challenges. While these tumors are benign, rare cases of tumor embolism to the brain have been reported. Surgical resection remains the treatment of choice, with excellent long-term outcomes in most cases.</p> Case presentation <p>A 27-year-old woman presented with progressive palpitations, shortness of breath, chest pain, and cough. Transthoracic echocardiography revealed a large, mobile mass in the left atrium. Surgical excision was performed, revealing a gelatinous tumor with areas of glandular differentiation. Immunohistochemical analysis confirmed the epithelial origin of the glandular structures, with a diagnosis of cardiac myxoma with glandular differentiation.</p> Conclusion <p>Cardiac myxomas with glandular differentiation are rare but important entities that require careful histological and immunohistochemical evaluation to avoid misdiagnosis as primary or metastatic adenocarcinoma. Patients should be monitored for recurrence and potential embolic complications.</p>