Primary pulmonary epithelioid angiosarcoma is a rare tumor type without any specific clinical and imaging features. Therefore, it is associated with high rates of misdiagnosis. The present study reports the case of a 54-year-old female patient who was admitted after complaining of cough, expectoration and bloody sputum for >5 months in May 2021. The patient reported a previous history of papillary thyroid carcinoma in 2003 and had undergone treatment through surgery, postoperative chemotherapy and iodine131 therapy. Chest computed tomography (CT) was performed in May 2021, which indicated that the disease had progressed rapidly since February 2021. CT-guided lung biopsy and immunohistochemical staining of the tumor indicated positivity for CD31, CD34 and E26 transformation-specific-related gene markers. The tumor was negative for thyroid cancer-associated antibodies; thus, a diagnosis of primary pulmonary epithelioid angiosarcoma was made. The patient died 3 months after the diagnosis. Primary pulmonary epithelioid angiosarcoma is a rare tumor type with high recurrence and metastasis rates. This tumor has no specific clinical symptoms and signs and is thus easily misdiagnosed. Biopsy is essential for diagnosis of the disease, particularly if patients have a tumor history.