Due to the rarity of pulmonary EHE, there is no clear standard of treatment in the absence of well-defined clinical trials. Partial spontaneous regressions have been described and watchful waiting has been reported to be an acceptable option, particularly in asymptomatic patients. Progressive or symptomatic disease may require treatment. Surgical resection may be performed for local disease control in cases of unilateral disease; but often, this is not an option because the presentation is, typically, bilateral and multifocal at diagnosis. Systemic therapy options have been poorly studied in EHE. Although the outlook can be grim, patients with poor prognostic markers or a more aggressive course may benefit from chemotherapy as suggested by a small case series that demonstrated some regression of tumor size and symptoms after treatment with (or a combination of) carboplatin, paclitaxel, bevacizumab, thalidomide, and α-interferon. Given the endothelial origin of EHE and in the absence of active bleeding, the antagonists of vascular endothelial growth factor may potentially play a significant role in the treatment of EHE.