The clinical data of 2 cases of myxoid adrenocortical adenoma (MACA) with a pseudoglandular pattern were collected and followed up. One of them is male, the other is female. Aged 53–56 years old. Separated occurred on left and right adrenal. Grossly, the mass was mostly solid, roundish and with clear boundaries, cutting indicates greyish white to local translucence, no obvious hemorrhage and necrosis. At light microscopy, tumor was demarcated by a fibrous capsule, atrophic adrenal gland tissue was found at the periphery of the tumor. Tumor cells arranged in compact cords and tubules, while other cells formed pseudoglandular patterns, floating loosely in the myxoid background. The neoplastic cells were small to medium size, polygonals with well-defined cellular borders, the cytoplasm was abundant eosinophilic or clear, the nuclei was round or oval, nucleoli was clear. The tumor showed limited dysplasia or cell pleomorphism, rare mitoses and no evidence of necrosis or vascular invasion. Immunohistochemistry showed that tumor of 2 cases were positive for Vimentin, Melan-A, CD56, NSE, one of them was positive for synaptophysin and alpha-inhibin; the other one show CK foci positive; While both were negative for EMA, HMB45, S-100, chromogranin-A, P53, EGFR, Ki-67 index were 1%. Special histochemical staining for Alcian-blue PAS highlighted the abundant eosinophilic myxoid material in the glandular cavity and interstitial. MACA with a pseudoglandular pattern was a extremely rare tumor of adrenal neoplasm, accurate diagnosis should combine clinical characteristics, CT, MR imaging with pathological features. Meanwhile, MACA should be distinguished from metastasis adenocarcinoma and other retroperitoneal myxoid degeneration of tumors.