| Summary: | We report a case of cutaneous adenoid cystic carcinoma arising on the left supraorbital region in a 65 -year-old woman. No primary was detected elsewhere in the body. Initial histopathology examination had reported it to be a basal cell carcinoma. The patient was treated with wide local excision and reconstruction with local flaps, followed by adjuvant radiotherapy. The patient remained tumor-free at 24-month follow-up. Till date, 64 confirmed cases of PCACC have been reported in detail in the English literature along with one population based study from the United States, which described 152 cases of PCACC diagnosed over a period of 30 years. Cutaneous adenoid cystic carcinoma has potential for distant metastasis as opposed to basal cell carcinoma so strict vigilance has to be maintained in post-operative period. We propose that possibility of such tumor should be kept in mind during treating skin malignancies such as basal cell carcinomas. Also wide local excision followed by adjuvant radiation is an ideal method to achieve margin-free removal of cutaneous adenoid cystic carcinoma and prevent distant metastasis. © 2018 The Authors
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