Pulmonary EMH is uncommon. Analysis of the reported cases shows slight female predominance with a median age of 60 years. Diagnosis is made with the help of clinical, radiographic, and histologic findings. Clinically, patients with pulmonary EMH may present with dyspnea on exertion, progressive decline in exercise tolerance, cough, hemoptysis, and fatigue. Physical examination may reveal coarse rhonchi and inspiratory crackles. Bilateral interstitial infiltrates, reticulonodular opacities, hilar prominence due to adenopathy, and bilateral pleural effusion may be seen on chest radiograph. CT scan of the chest is helpful in making the diagnosis of pulmonary EMH. Findings on CT scan of the chest in a limited number of previously reported cases of pulmonary EMH include bilateral nodular lesion with septal thickening, patchy consolidation with or without bilateral pleural effusion, and patchy or diffuse ground glass appearance. A number of radiotracer scans have been used to demonstrate EMH and the most widely used is 99mTc-colloid. Although a widely accepted technique in the past to noninvasively supplement the diagnosis of EMH, false-negative results have been reported. The diagnosis of pulmonary EMH typically requires tissue diagnosis, which can be obtained by a surgical lung biopsy. Lung parenchymal samples obtained via transbronchial biopsy in the case of diffuse parenchymal disease may also be adequate to confirm diagnosis. Imaging-guided fine needle aspiration has also been used with success in cases of nodular lesions. Histologically, pulmonary EMH is characterized by focal or diffuse intravascular, luminal, alveolar, peribronchial, perivascular, and interstitial involvement by foci of trilineage hematopoietic elements. Fibrosis may or may not be present in these areas. Immunohistochemical staining for myeloid, erythroid, and megakaryocyte precursors is required on lung parenchymal specimens. Immunohistochemical stain for Factor VIII highlights the megakaryocytes, which stain the cytoplasm dark brown. To date there have been no reported cases of BAL demonstrating hematopoietic elements in EMH. Appropriate clinical setting along with radiologic features should raise the suspicion for this entity and the diagnosis should be confirmed by tissue sampling.