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The purpose of this review is to present a summary of the diagnosis, prognosis, and treatment of CTCL, with specific emphasis on mycosis fungoides (MF) and S\u00e9zary syndrome (SS). We also present a detailed discussion of the entities that make up the differential diagnosis of CTCL.<\/jats:p><jats:p>DESIGN: We reviewed the medical literature on CTCL and other diseases that make up the differential diagnosis of CTCL.<\/jats:p><jats:p>Results and<\/jats:p><jats:p>CONCLUSION: MF and SS are the most common forms of CTCL. The etiology of this disease is still unknown. Patients may go for months to years with skin abnormalities before being diagnosed. MF\/SS is an indolent disease and patients with T1 disease have a normal life expectancy. Patients who undergo transformation to large-cell lymphoma (8% to 23% of patients) have a poor prognosis, with mean survival ranging from 2 to 19 months. Treatment for MF\/SS continues to be palliative. 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