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Cutaneous T-cell lymphoma is a heterogeneous group of non-Hodgkin's lymphoma, characterized by an infiltration of malignant T cells in the skin. The most common subgroups include mycosis fungoides followed by the aggressive leukaemic variant, Sézary syndrome. The pathophysiology of this neoplasm is poorly understood. The diagnosis of mycosis fungoides at the early stages can be challenging due to phenotypic similarities with other skin conditions. A lack of understanding of the aetiopathology of this neoplasia makes prognosis and diagnosis, as well as the development of targeted therapies aimed at long remission, challenging. This review provides an update on the aetiopathology of cutaneous T-cell lymphoma with regards to genetic and epigenetic alterations, current diagnostic tools and treatments, as well as emerging therapies.