Introduction: This is a report of the histopathological characteristics of a rare case of primary adrenal Myxoma. A 25-year-old male was admitted to AI-Zahra Hospital due to abdominal trauma. Radiological evaluations revealed an adrenal mass without any clinical manifestation. The MRI of abdomen showed a well-defined cystic mass of right adrenal with the following differential diagnoses: cystic adenoma, cystic pheochromocytoma and metastatic lesions with cystic degeneration. Right adrenalectomy was performed and in pathologic study, the mass was grossly solid and had yellow-grayish color and gelatinous consistency in cut surface. Microscopically, it was composed of myxoid stroma with few scattered cells which were positive for vimentin but negative for CK, CEA, S100, Desmin, Actin and Melan-A in immunohistochemical staining. These findings are characteristic of primary adrenal Myxoma.Discussion: Pathologically, it may be difficult to differentiate Myxoma from other malignant tumors with a myxoid stroma. Further studies are necessary to clarify the origin and histogenesis of this lesion.