The authors report a case of 50-year man who presented with 2-year history of dry cough, right sided chest pain, and shortness of breath. Chest X-ray revealed right-sided pleural effusion and left-sided opacity. Pleuroscopic pleural biopsy confirmed the diagnosis of primary pleural epitheliod hemangioendothelioma (EHE) with peripheral lung parenchymal invasion. Chest drain was inserted; and significant amount of fluid was drained, but lung failed to expand after 72 hours. Patient was planned for video assisted thoracoscopy (VATS) and also discussed with oncology department for chemotherapy; but he refused any further treatment, and left home against medical advice with chest drain in place. EHE originating from pleura is extremely rare with an aggressive clinical course and poor prognosis. To our knowledge, this is the first reported case of an EHE originating from pleura in South Asia and highlights the heterogeneous geographic distribution of tumor and demonstrates the need for a more systemic approach to all patients with unilateral pleural effusion.