Porocarcinoma is a rare tumor of the eccrine sweat glands that usually disseminates to the regional lymph nodes, but it can also develop distant metastasis. Case presentation: We report the case of a 67 year-old female patient who underwent wide surgical resection of a left cervical cutaneous tumor in a primary care center, for which the histology exam of the specimen was mixed basal cell and squamous cell carcinoma. She was referred to our hospital's oncology clinic and histologic re-evaluation changed the diagnosis to eccrine porocarcinoma (EPC). Computer-tomography (CT) revealed cervical lymphadenopathies for which the patient underwent 4 cycles of chemotherapy, without regression. She subsequently underwent a left upper anterior jugular lymphadenectomy (group IIa) with all nodes being negative and, three months later, she developed a unique adenopathy under the parotid gland that was excised and confirmed to be metastatic. Postoperative external radiotherapy was administered with a good outcome on CT scan. Nine months after her last surgery, the patient did not show any sign of recurrence or distant metastasis. Conclusion: EPC is a challenge, both diagnostically and therapeutically. In the absence of consensus regarding the indications and extent of lymphadenectomy and adjuvant therapy, patients with EPC should be referred to an experienced multidisciplinary team in a tertiary center.