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Introduction: Syringocystadenocarcinoma papilliferum (SCACP) is a rare malignancy of the elderly originating from apocrine adnexal glands, mostly developing in the head and neck district. To date, there is no established treatment for metastatic SCACP, leaving affected patients with small therapeutic opportunities. Case Presentation: Herein, it is described the case of a 70-year-old man affected by metastatic SCACP, treated with three different sequential therapy lines: carboplatin plus paclitaxel, epirubicin, and an off-label protocol with enzalutamide, achieving 41 months overall survival. Off-label enzalutamide treatment has been justified by androgen receptor expression by SCACP cells, a common feature of apocrine glands derived tumors. Interestingly, SCACP demonstrated to be responsive to enzalutamide treatment, even though the patient precociously stopped the treatment due to severe toxicity. Conclusion: This case represents the first evidence of hormonotherapy administration in a SCACP patient, possibly providing the base for a new therapeutic opportunity for this rare malignancy.