<p>Sweat gland carcinomas constitute a heterogeneous group of malignant neoplasms with differentiation towards the sweat gland apparatus that may arise de novo or from the transformation of a precursor. Over the last few years, a number of specific genetic alterations have been identified allowing a more accurate classification of this diverse group of neoplasms. In the present review, we focus on adnexal sweat gland carcinomas arising de novo including microcystic adnexal carcinoma, squamoid ductal eccrine carcinoma (SDEC), Endocrine mucin-producing sweat gland carcinoma and mucinous carcinoma, digital papillary adenocarcinoma, adenoid cystic carcinoma, apocrine carcinoma, secretory and microsecretory carcinoma and NUT adnexal carcinoma. The main clinical, morphological, immunohistochemical and molecular features of these tumour entities are described and the main differential diagnoses are discussed.</p>