Primary cutaneous lymphomas are composed of both T-cell (75%) and B-cell lymphomas and are rare conditions representing 2% of all lymphomas with an annual incidence of 0.3 to 1 per 100 000. MF was first described by Alibert in 1806 as a mushroom like neoplastic skin condition. It most often presents in those aged 45 to 60 years but has been diagnosed in children and adolescents. It is 50% more common in black than in white patients and twice as frequent in men as in women. Treatment of early-stage disease (stage IA-IIA) typically involves skin directed therapies that include topical corticosteroids, phototherapy (psoralen plus UVA or UVB), topical chemotherapy, topical or systemic bexarotene, and radiotherapy. Systemic approaches are used for recalcitrant early-stage disease, advanced-stage disease (stage IIB-IV), and transformed disease and include retinoids, such as bexarotene, interferon, histone deacetylase inhibitors, the fusion toxin denileukin diftitox, systemic chemotherapy including transplantation, and extracorporeal photopheresis.