Abstract: A case of primary cutaneous adenoid cystic carcinoma ( PACC) of the scalp was reported and the related literature were reviewed. A  44-year-old female, presented, nodules on the overhead for 10 years with pain for 1 year. Histopathological examination showed the tumor  was located in the dermis with clumpy clumps clustered and distributed. There were many tubular and cystoid structures in the center of the clumps, and mucin deposits were visible in the cystoid, forming a sieve structure. The tumor cells were single basophil cells with dense nuclei, not obvious nucleoli, light cytoplasm, and mitotic astigosis. Immunohistochemistry results showed CD117 (+), CK7 (+), CK5/6 (+), S-100 (focal +), P63 (+), SOX-10 (+), GCDFP15 (-),  Ki-67 positive cells accounted for about 20%. Surgical resection was performed with no recurrence after two years of follow-up.

Key words: adenoid cystic carcinoma, primary