Clear cell hidradenocarcinoma (CCH) is an exceedingly rare and highly malignanttumor of the eccrine sweat glands.Its treatment is extremely difficult due to the characteristically aggressive clinical course including repeated local recurrence and uncontrollable distal metastasiscoming along with a very poor prognosis.Most published case studies recommend a wide surgicalexcision followed by adjuvant conservative therapy,which is generally considered to be thestandard treatment.Two cases of nodular CCH of the scalp either presenting as a singular primarylesion or at an already metastatic stage were analyzed retrospectively.Wide local excision of thetumor couldnt prevent the primary carcinoma from recurring and metastasizing.Both casesreceived various therapies but the results were unsatisfactory.Although most authors haverecommended that early wide surgical excision of the tumor is a feasible therapeuticmeasurement,our results raise doubts on the efficacy of this treatment strategy.As alternativeapproaches (i.e.chemotherapy,radiotherapy) are similarly controversial,further studies anda wide exchange of clinical experiences are crucial.