This report describes a case of pulmonary epithelioid haemangioendothelioma presented as multi-organ metastases including lung, liver and bone in a 56-year-old woman with an initial diagnosis made with thoracoscopic wedge biopsy. The diagnosis is confirmed through immunohistochemistry. This is a rare disease, with approximately 90 cases described in the English literature and approximately 10 cases reported in China. The case of PEH presented as multiple pulmonary nodules and metastasing to liver and bone is rare in the English literature. The rarity of this condition, the lack of clear standards for treatment, and the partial-to-complete spontaneous regression of EHE seen in some patients up to 15 years from initial detection makes it difficult to decide on the most appropriate treatment. This report may contribute to the data on clinical findings and natural history of this rare tumor.