Background: Cutaneous T-cell lymphoma (CTCL) represents a spectrum of diseases composed of malignant helper T lymphocytes. An accurate diagnosis of early CTCL is difficult because of the varied clinical and histologic expressions of the disease. Methods: The authors review the epidemiology, possible risk factors, clinical manifestations, diagnostic techniques, staging, prognosis, and treatment options for CTCL. Results: The varied and often nonspecific clinical and histologic presentations of CTCL may delay diagnosis and staging, thus necessitating further studies such as immunophenotyping flow cytometry, and T-cell receptor gene rearrangement analysis. Conclusions: A multidisciplinary approach to the diagnosis, staging, and treatment of CTCL assists in optimizing outcomes from management of patients with this disease.