We conducted a clinicopathologic study of adenoid squamous cell carcinoma involving the eyelid (15 cases) and periorbital region (six cases). The mean age of the 17 men and four women was 64.5 years (range, 43 to 85 years). Histopathologically, the lesions were characterized by acantholysis and a pseudoglandular pattern with many alveolar and tubular structures. Solar keratosis was also a prominent histologic feature, suggesting that exposure to the sun was a predisposing factor. Local recurrences occurred in five of 16 patients for whom follow-up information was available (mean follow-up period, 8.8 years). There was no regional lymph node or distant metastasis, and none of these patients died of their neoplastic disease. Surgical excision appears to be the best treatment.