Anaplastic large cell lymphoma (ALCL) is a rare type of non-Hodgkin lymphoma (NHL) and it originates from mature T-cells. The contemporary WHO Classification of Haematologic Malignancies recognizes primary cutaneous ALCL (PC-ALCL), two subtypes of systemic ALCL (ALK-positive and ALK-negative) and breast implant-associated anaplastic large cell lymphoma (BIA-ALCL) as a newly included CD30+ pathological entity. PC-ALCL is classified as one of the primary cutaneous T-cell lymphoproliferative disorders. ALK-positive ALCL usually affects children and young adults, whereas ALK-negative ALCL is more common in patients over the age of 55 years. Both systemic lymphomas are treated as aggressive lymphomas, but the disease course may be different. ALK-positive ALCL responds well to standard chemotherapy treatments, putting most patients into long-term remission. ALK-negative ALCL initially responds to treatment as well, but the disease is more likely to relapse. BIA-ALCL is a distinct form of CD30-positive and ALK-negative ALCL that arises in association with a breast implant (usually 8-10 years after surgey). Despite the low incidence of this new disease, the increasing use of breast implants for cosmetic or post-mastectomy reconstruction purposes places BIA-ALC as an emerging and compelling medical challenge. The etiopathogenesis of BIA-ALCL is likely to be multifactorial, with current evidence-based theories recognizing the combination of chronic inflammation, biofilm formation, implant texturization, genetic predisposition and time. Breast implants with textured surfaces seem to be associated with nearly all cases of BIA-ALCL. Late onset, persistent seroma around breast implant represents the classical clinical presentation. Most of the patients present with localized disease, which confers an excellent prognosis. Complete surgical excision has a key role in the treatment.