Primary cutaneous adenoid cystic carcinoma is a rare slow-growing neoplasm, with limited literature reporting the involvement of the scalp. It has a tendency to recur locally; however, lymph node, distant pulmonary and bony metastases are exceptionally rare. We highlight the case of a 65-year-old female with primary cutaneous adenoid cystic carcinoma with distant pulmonary and bony metastases and the importance of imaging in diagnosing distant metastasis and perineural spread.