Purpose To report the clinicopathologic features of a solitary superficial angiomyxoma arising in the eyelid. Design Case report. Methods A retrospective review of the clinical and pathologic features of a patient with solitary superficial angiomyxoma in the eyelid. Results A 47-year-old male presented with a right upper lid mass for 6 months. Excisional biopsy was performed, and microscopic examination revealed a tumor comprising loose spindle or stellate-shaped cells in myxoid stroma sprinkled with small numbers of neutrophils. The tumor cells were negative for smooth muscle actin, desmin, S-100 protein, and CD34 on immunostaining. Conclusions Ophthalmologists should be aware of superficial angiomyxoma as a rare cutaneous tumor with a tendency for local recurrence. Multiple lesions and occurrence in the external ear can be associated with the Carney’s complex.