Introduction: Eccrine spiradenoma is a rare benign tumour of sweat gland origin. It often presents as a slow-growing, nodulo-cystic lesion on the extremities and is rarely found on the scalp. On histopathology, we diagnosed an eccrine spiradenoma in a 50-year-old.Materials and methods: A systematic review of all available literature from the Cochrane Library, MEDLINE and PubMed was conducted on cases of eccrine spiradenomas of the scalp. Data on demographics, size of lesion, duration prior to diagnosis, histology and immunocheistry, treatment and follow-up were reviewed. We also present our case report.Results: There are 4 documented cases of pure eccrine spiradenoma of the scalp in our review. The mean age at presentation was 36 years. The mean size of the tumour was 15 mm with an equal incidence in males and females, along with a predominant occurrence in the Caucasian population. There were no recurrences following the primary excision of the lesion and no malignancy was found on histology.Conclusion: Eccrine spiradenomas may present as a diagnostic challenge. Their malignant potential, albeit small, means they should be considered in any solitary cystic scalp lesion. To our knowledge, this is the first documented case of pure eccrine spiradenoma of the scalp in the United Kingdom.