PURPOSE: Adenoid cystic carcinoma (ACC) is a rare malignant epithelial neoplasm derived from the salivary glands. In some cases, ACC may arise in other primary sites, such as skin. We report a case of adenoid cystic carcinoma arising the scalp skin of a 69-year-old woman. METHODS: A 69-year-old woman presented with a tender scalp nodule. A local wide excision was performed. Histopathologic examination revealed the adenoid cystic carcinoma with basaloid cells in a cribriform pattern. The resection margins were free of tumor. Two years later a tumor recurred in the scarred area. The lesion was removed surgically and the histopathological diagnosis of adenoid cystic carcinoma was again established. After two years, tumor recurred again and distant metastasis of the lung was diagnosed. A surgical wide excision was done and the close regular follow-up for recurrence was done. Two years later, third recurrence of the scalp was observed. We also performed the wide local excision with tumor free margin. RESULTS: We experience the recurrent adenoid cystic carcinoma of the scalp with pulmonary metastasis. We have performed the wide local excision for three times. The patient has been followed up for 10 years with regular work-up for recurrence and metastasis CONCLUSION: primary cutaneous adenoid cystic carcinoma is a rare skin neoplasm with a high potential for recurrence after local excision. The standard treatment of ACC is wide local excision with tumor-free margins established by permanent section.