To report a case of intravascular papillary endothelial hyperplasia of the eyelid.

A 30-year-old male presented with a right upper eyelid mass, which had been growing in size for three years. The mass was about 7 mm in diameter, purplish, spherical, and cystic. Incision and drainage were performed, but the cystic mass instantly refilled with blood. Excisional biopsy was performed. On microscopic examination, a myriad of small delicate papillae projections into the dilated vascular lumen with organizing thrombus were noted. Each papilla was lined with a single layer of endothelial cells, surrounding a collagenized core. The endothelial cells were reactive for CD31 on immunohistochemical staining. There were focal areas of frequent mitoses, but neither cytological atypia nor necrosis was found. Hence, the lesion was diagnosed as intravascular papillary endothelial hyperplasia.

Intravascular papillary endothelial hyperplasia in the periorbital area is rarely reported, and it is important to distinguish it from hemangioma or angiosarcoma. Complete surgical excision is necessary to prevent recurrence. The authors report a case of intravascular papillary endothelial hyperplasia of the upper eyelid, which should be considered in the differential diagnosis of eyelid or orbital tumor.