Epithelioid hemangioendothelioma (EHE) is a very rare vascular neoplasm that is often asymptomatic. A 40-year-old woman presented to the emergency department for evaluation of a nonproductive cough, chest pain, and dyspnea. A chest computed tomography angiography (CTA) demonstrated necrotizing pneumonia, a loculated left-sided pleural effusion, and an acute pulmonary embolus. She was started on broad-spectrum intravenous antibiotics and heparin infusions, and a chest tube was placed. After minimal improvement in her pleural effusion following instillation of fibrinolytics, she underwent video-assisted thoracoscopic surgery with decortication, and a pleural biopsy was performed. Her presenting symptoms resolved shortly thereafter. Following discharge, surgical pathology resulted in a diagnosis of EHE. She was not a candidate for surgical resection and remained under surveillance. A year later, she was found to have metastatic disease, and radiotherapy was initiated. Our case, which presented as necrotizing pneumonia associated with pulmonary EHE, highlights the challenges in diagnosing this disease given its extreme rarity and discusses its management.