A case of primary myxoma of the lung is presented. The patient is a 70-year-old woman who presented with a history of dyspnea, cough, and mild chest discomfort. Chest radiographic studies disclosed the presence of a well-marginated nodule in the left upper lobe. A wedge resection of the tumor mass was performed. The specimen contained a tan-gray, soft, well-circumscribed tumor nodule of approximately 1.8 cm in greatest dimension. Histologically, the tumor was characterized by a well-demarcated lesion, composed of loose spindle and stellate cells embedded in a myxoid matrix, containing thin-walled vessels. Immunohistochemical studies showed the cell to be negative for S-100, keratin, desmin, smooth muscle actin, and HMB-45. Vimentin stain showed positive staining in some of the stellate cells. No evidence of recurrence or metastatic disease was observed after 18 months.