Cilj: Cilj je ovog rada prikazati slučaj vrlo rijetkog malignog tumora glave i vrata – malignog ekrinog spiradenoma, opisati kliničke i patohistološke karakteristike tumora, važnost rane dijagnoze s obzirom na konačni ishod te metode liječenja. Prikaz slučaja: U radu je opisan slučaj 46-godišnje pacijentice kojoj je dijagnosticiran hemangiom lijeve strane vrata te se 25 godina kontrolirala. Nekoliko mjeseci prije javljanja na Kliniku tvorba se naglo počela mijenjati. Na temelju ultrazvuka vrata, citopunkcije, magnetske rezonancije vrata i biopsije postavljena je sumnja na metastazu nepoznatog primarnog tumora. PET/CT-om nije otkriveno sijelo primarnog tumora te je tek kirurškim zahvatom i patohistološkom verifikacijom operacijskog preparata utvrđena definitivna dijagnoza malignog ekrinog spiradenima s metastazom u jednom regionalnom limfnom čvoru vrata. Pacijentica je poslijeoperacijski tretirana radiokemoterapijom. Šest mjeseci nakon liječenja utvrđene su multiple metastaze pluća, pleure, medijastinuma i jetre, pa je 3 mjeseca kasnije nastupio smrtni ishod. Rasprava i zaključak: U većini slučajeva ovaj rijedak tumor nastaje malignom transformacijom benignog ekrinog spiradenoma, premda može nastati i de novo. Rijetko se javlja u regiji glave i vrata. Dijagnoza se temelji na patohistološkom nalazu. Tumor pokazuje veliku sklonost lokalnim recidivima te regionalnim i udaljenim metastazama kada je prognoza loša. Liječenje je prvenstveno kirurško, dok je učinak radiokemoterapije upitan. Važno je bolest dijagnosticirati u ra- noj fazi, kada ne postoji regionalna i udaljena proširenost jer je tada stopa izlječenja vrlo visoka., Aim: To present a case of a very rare malignant head and neck tumor – malignant eccrine spiradenoma, describe the clinical and pathological characteristics of the tumor, as well as the importance of early diagnosis with respect to the ultimate outcome of these treatments. Case report: This paper describes the case of a 46-year-old patient who was di- agnosed with hemangioma of the left side of the neck, and was followed for 25 years. Few months before the patient came to the Clinic, the lesion suddenly began to change. Based on the neck ultrasound, fine-needle cytopuncture, MRI and biopsy a suspicion on a metastasis of unknown primary tumor was established. PET / CT has not discovered the location of the primary tumor and only surgery and histopathologic verification of operating sample established the definitive diagnosis of malignant eccrine spiradenoma with metastasis in a single regional lymph node. The patient was treated with postoperative radio- and chemotherapy. Six months after the treatment, multiple metastases of the lung, pleura, mediastinum, and liver were found and 3 months later fatal outcome occurred. Discussion and conclusion: In the majority of cases this rare tumor is caused by malignant transformation of benign eccrine spiradenoma, although it can appear de novo. It rarely occurs in the head and neck region. Diagnosis is based on pathohistological findings. The tumor shows a strong preference to local and regional recurrence and to distant metastases, the prognosis of which is poor. It is important to diagnose the disease at an early stage when there is no regional or distant spread because at that stage the survival rate is very high.