We report a case of a 65-year-old man with metastatic primary cutaneous adenoid cystic carcinoma that was effectively treated by combination chemotherapy with cisplatin (CDDP) plus vinorelbine(VNR). He detected a tumor mass on the anterior surface of his left patella in 2003 and underwent a tumorectomy in October 2008. He was given a diagnosis of primary cutaneous adenoid cystic carcinoma with metastatic lesions to multiple lungs and left tibial bone and then given chemotherapy combining CDDP plus VNR as the first treatment in December 2008. By this treatment for six cycles, the lung metastatic tumors gradually reduced on chest CT. We reported the efficacy of combined treatment with CDDP plus VNR for primary cutaneous adenoid cystic carcinoma, because this clinical condition was very rare and the standard treatment has still not been established.