Endometrial stromal tumours (ESTs) are diagnosed in most instances by light microscopy. Often, the greatest challenge is to distinguish between the different subtypes of these tumours. Furthermore, a handful of new or relatively new entities have been described in the literature, which may cause problems in the differential diagnosis; highly cellular leiomyoma is the most common. In addition, new antibodies have been developed to help in the distinction of ESTs from their mimics, as there are prognostic and therapeutic implications. A practical approach is provided for the diagnosis of ESTs on the basis of systematic assessment of histological and immunohistochemical parameters, and recent developments related to these tumours are highlighted.