Primary cutaneous lymphoma is a rare heterogenous group of extranodal non-Hodgkin's lymphomas of which primary manifestation involve the skin. Primary cutaneous lymphoma consists of two major entities: cutaneous T-cell lymphoma and cutaneous B-cell lymphoma, each further divided into common subgroups. Cutaneous T-cell lymphomas account for the majority of diagnosed cases, with mycosis fungoides and Sézary syndrome being its most important entities. The etiopathogenesis of primary cutaneous lymphoma is relatively unknown and poorly understood. However, recent breakthroughs in research have established an involvement of abnormal inflammatory stimulation and expression of various cytokine cascades, driving tumorigenesis. Given the significant overlaps and resemblances of primary cutaneous lymphoma subtypes in clinical features and immunohistopathological findings, a thorough and comprehensive assessment process is required to correctly diagnose the nature of the tumor, determine the prognosis of the disease, and initiate accurate treatment. Primary cutaneous lymphomas, as rare diseases, require a multidisciplinary approach in diagnosis and treatment. The main goal of treatment in these patients is to improve the quality of live while avoiding diverse toxicities.