Parotid masses are common in our clinical practice, with three-fourths of the cases being benign lesions and pleomorphic adenomas having the highest incidence. In contrast, angiosarcoma is a rare malignant neoplasm that accounts for <0.1% of all head and neck cancers. Cutaneous angiosarcoma is the most common subtype that involves the head and neck region and presents initially as bruise-like patches. Angiosarcoma progresses rapidly, with a mortality of 50% within 3 years from diagnosis even after surgical resection. We report a case of parotid angiosarcoma in a 69-year-old woman that initially presented as left preauricular and postauricular tumors for 1 month. Definitive diagnosis was made using immunohistochemical staining of the excised tissue. Disease severity was assessed using chest computed tomography and a whole body gallium scan, which suggested an inactive disease. The patient responded to surgical excision and postoperative radiotherapy extremely well. She is under regular follow-up postoperatively, with no evidence of recurrence. This case helps improve our understanding of the diagnosis and treatment of angiosarcoma, given its nonspecific presentation and poor prognosis owing to consequent late diagnosis. (J Taiwan Otolaryngol Head Neck Surg 2022; 57:123-128)
