Primary cutaneous adenoid cystic carcinoma (PCACC) is a particularly rare variant of sweat gland carcinoma with characteristics of indolent and progressive course and high incidence of perineural invasion and local recurrence. However, regional lymph node metastasis in PCACC is exceedingly rare and its prognostic implication is unknown. Only two previous cases of recurrent scalp PCACC were reported to be associated with cervical lymph node metastases at 42 months and 20 years, respectively, after the initial treatment. We present a case of PCACC occurring in the left parotid region with regional neck lymph node metastasis in a 64-year-old man. Because the occurrence of lymph node metastasis seems to be associated with recurrent PCACC, we suggest that neck dissection may be included in the treatment for recurrent PCACC patients.