Cutaneous angiosarcomas mainly arise on the scalp and face of older patients. They present with reddish nodules or plaques and tend to bleed and ulcerate. Angiosarcomas show an aggressive and recurrent course and have a high potential for metastasis. We report on a 48-year-old patient with unilateral eyelid edema as the first manifestation of an angiosarcoma. The clinical presentation, the history of contact allergy, the presence of antinuclear antibodies, and abnormal thyroid function tests favoured a non-neoplastic disease. However, multiple biopsies confirmed the diagnosis of a highly differentiated angiosarcoma. Staging of the patient revealed no metastasis. After radical surgery including orbital exenteration and postoperative radiation therapy, no recurrence was observed with a follow up of 26 months. This case underlines the importance of including angiosarcoma in the differential diagnosis of unilateral eyelid edema.