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[A primary adenomucinous carcinoma on the upper eyelid].

In this case we present a 70-year-old male with a primary adenomucinous carcinoma (PKMK) on the upper eyelid. Radical surgery or radiotherapy could not be implemented, due to aggressive behaviour caused by dementia. PKMK is a rare tumour with 228 described cases. Clinically the tumour can be misdiagnosed as benign, but has the potential to lymph node-, regional- and remote metastasis. Histologically it may be difficult to distinguish from adenocarcinomas (breast-, colorectal-, ovarian-, prostate- and lung cancer). The treatment is surgical with radical excision by Mohs' procedure (abroad) or guided by intraoperative biopsies (Denmark).