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A 65-year-old woman presented with a 6-month history of chest tightness, non-productive cough and dull chest pain. She had no significant occupational or smoking history. Laboratory findings were unremarkable. Chest CT (figure 1A–C) demonstrated diffuse left pleural nodular thickening, a large haemorrhagic pleural effusion (confirmed by diagnostic thoracentesis) and consequent passive left lung atelectasis. Pleural fluid cytology was negative for malignant cells. Due to diagnostic uncertainty, she underwent video-assisted thoracoscopic surgery, which revealed diffuse, nodular pleural thickening and haemorrhagic fluid, followed by open decortication. Histopathology (figure 1D) revealed nests of epithelioid cells with eosinophilic cytoplasm and mild nuclear atypia, embedded in a myxohyaline stroma. Immunohistochemistry (figure 2A and B) showed positivity for vascular endothelial markers (CD31, CD34, Fli-1, ERG) and the lymphatic marker D2-40, while epithelial (cytokeratin) and mesothelial (calretinin, WT-1) markers were negative. The Ki-67 proliferation index was approximately 8%. Critically, fluorescence in situ hybridisation detected a WWTR1-CAMTA1 gene fusion, confirming the diagnosis of pleural epithelioid haemangioendothelioma (PEHE). Given the absence of a standard treatment protocol for this rare malignancy, a multidisciplinary team decision was made to administer systemic chemotherapy. After three cycles of carboplatin and etoposide, restaging CT showed partial regression of the pleural lesions. However, the disease pursued an aggressive course. Three years post-operatively, the patient developed new back pain, and imaging revealed widespread metastases to both lungs and multiple vertebrae. Despite palliative radiotherapy, she died 3.5 years after her initial diagnosis.