• Intravascular papillary endothelial hyperplasia (IPEH), a benign lesion histopathologically characterized by intravascular fibrous papillary fronds covered by pleomorphic endothelium, is usually found in the subcutaneous tissues of the head and extremities. We describe the clinical and histologic findings of IPEH in the upper eyelid of a 20-year-old woman. Intravascular papillary endothelial hyperplasia has been confused with angiosarcoma. The differential diagnosis and key distinguishing features are discussed and the literature is reviewed.