The objective of this review is to summarize and evaluate the current knowledge of chronic progressive lymphoedema (CPL) in draught horses. Clinical signs of this multifactorial disorder are mainly restricted to the lower limbs, comprising progressively deteriorating skin swelling and deformation. Although typical lesions were first reported in the beginning of the 20th century, CPL was only recognised as a specific syndrome in 2003, and since then research has driven forward. Despite the high prevalence in some breeds and the serious economic impact, the pathogenesis is not fully understood and the available treatment options remain symptomatic and non-curative. There is a need to improve diagnostic techniques and to develop selection tools.