A 7 cm diameter tumor of the left atrium is reported in a 78 year-old woman with a past history of pT3N1M0 colonic adenocarcinoma. The histological examination of the atrial tumor disclosed areas of highly vascularized myxoid stroma with cells strongly reactive for vimentin. Multiple mucoid spaces lined by a single layer of goblet cells were scattered among those typical areas of myxoma. No nuclear atypia was observed. Cytoplasm of the glandular cells was immunoreactive for epithelial antisera (keratin, EMA), CEA and CA19.9. Two years later, the patient was doing well, with no local recurrence of the cardiac myxoma and no secondary location of the colonic adenocarcinoma. The histological characteristics, the absence of atypia, the absence of tumoral extension or neoplastic lymphatic vascular thrombi in the pedicle or in the interatrial septum, and the finding of typical myxomatous areas supported the diagnosis of cardiac myxoma with glandular component. To our knowledge, 21 cases of myxoma with glandular mucinous component, focal or prominent, have been previously published in the literature. These myxoma were generally sporadic cases with the same clinical features and prognosis as typical myxoma. Immunoreactivity of these glandular structures was constant for epithelial markers. The positive immunostaining by CEA, and by CA19.9 in our case, reflects the histogenetic endodermal origin.