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BACKGROUND We present a case report of periocular squamous cell carcinoma and a review of the literature with emphasis on early diagnosis, proper follow-up and management, reconstructive options, and new immunomodulatory therapies.

OBJECTIVE The objective is to guide the dermatologist and the dermatologic surgeon in proper management and continued care of patients with periocular squamous cell carcinoma in light of its propensity for perineural involvement and regional lymphatic metastases.

MATERIALS AND METHODS A MEDLINE, Ovid, and PubMed search was conducted for recent relevant articles pertaining to “periocular,”“periorbital,”“squamous cell” carcinoma, and their “surgery”“treatment” modalities.

CONCLUSIONS Periocular squamous cell carcinoma is an aggressive tumor, characterized by perineural involvement and an overall rate of regional lymph node metastases reported to range from 10% to as high as 20% to 25%. Increased vigilance must be undertaken when treating these high-risk tumors. Mohs micrographic surgery or excision with frozen section analysis is the standard of care for periocular squamous cell carcinoma. Multiple options exist for the reconstruction of the postoperative defect that allow for excellent function and cosmesis. Finally, research into new immunomodulators will hopefully lead to an increased understanding of the aggressive nature of periocular squamous cell carcinoma and potential aid in the treatment of the tumor.