INTRODUCTION: This case-series is aimed to describe the natural history of epithelioid sarcoma (ES) and to provide insights into the differential clinical behaviour of its two variants ("classic-type" and "proximal-type"). The value of a subtype-adapted grading system based on pathological features is explored. METHODS: Data from consecutive, primary, localised, INI1-deleted ES operated at three Italian sarcoma reference centres (1995-2015) were included. Centralised pathological review was performed. Classic-type ES was broken down into "high-grade" and "low-grade", according to number of mitoses, evidence of necrosis and nuclear atypia. Five- and 10-year overall survival (OS) and crude cumulative incidence (CCI) of local recurrence (LR) and distant metastasis (DM) were estimated. RESULTS: Fifty-two patients were included. 5- and 10-year OS estimates were 707 577type ES, 774type ES (P = 0.02). In classic-type ES, 5- and 10-year OS was higher for low-grade (952 respectively) than high-grade tumours (P = 0.002). 5- and 10-year CCI estimates for LR were 213- and 10-year CCI estimates for DM were 359 both 28type ES, 479type ES (P = 0.03). CONCLUSIONS: Suffering from a proximal- or a classic-type is the stronger predictor of outcome in patients with localised ES, with proximal-type ES patients having lower survival due to a higher tendency toward metastatic spreading. However, the "high-grade" classic-type ES was associated with outcomes close to proximal-type ES.