Primary mucinous adenocarcinoma or mucinous eccrine carcinoma of the skin is a rare malignant neoplasm showing predilection to the head and neck. Distinguishing between these primary neoplasms and the more frequent metastatic mucinous deposits on the skin from primaries in the breast and gastrointestinal tract constitutes a diagnostic dilemma.We report a case of a 61-year-old lady who presented with a slow-growing, painless scalp nodule. Upon excision, it was diagnosed as “primary mucinous adenocarcinoma”. An extensive work-up in search for another primary tumour failed to show a primary malignancy elsewhere and the diagnosis of a primary eccrine mucinous adenocarcinoma of the skin was rendered.A review of the literature on this entity is presented, discussing diagnostic challenges and therapeutic options that of interest to surgeons, pathologists and dermatologists.These tumours are indolent and low-grade, with a tendency for local, sometimes multiple, recurrences. Proper patient counselling and follow-up are important in treatment. Sound collaboration between clinicians and pathologists, for good therapeutic results, is of utmost importance.