We report a 58-year-old woman with cutaneous adenoid cystic carcinoma arising on the chest treated with Mohs micrographic surgery. The patient remained tumor-free at 24-month follow-up. To date, only six other cases of cutaneous adenoid cystic carcinoma were reportedly managed by Mohs surgery. Cutaneous adenoid cystic carcinoma has low potential for distant metastasis but is notorious for its aggressive infiltrative growth pattern, frequent perineural invasion, and high risk of local recurrence after excision. We propose that Mohs surgery is an ideal method to achieve margin-free removal of cutaneous adenoid cystic carcinoma. A brief literature review is provided.