Primary pulmonary microcystic fibromyxoma is very rare. We describe a case of 68-year-old female who had a 4 months history of thorax suffocation and dyspnea. Computed tomography revealed a well-circumscribed nodule with a diameter of approximately 2.2 cm in the right upper lobe. After extensive workup, she received a right upper lobe wedge resection. Pathologically, microcystic architecture with myxoid stroma was revealed in the well-circumscribed tumor. The tumor cells were spindled to stellate, with innocuous appearance. Immunohistochemical staining showed strong expression of vimentin and CD99 in the tumor cells, but no expression of epithelial markers and other mesenchymal markers for distinctive differentiation. Genetically no EWSR1 or SS18 rearrangements were detected by fluorescence in situ hybridization. Eventually this case was diagnosed as primary pulmonary microcystic fibromyxoma. No tumor recurrence or metastasis was observed during 25 months follow-up.