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Breast implant–associated anaplastic large cell lymphoma (BIA-ALCL), a rare peripheral T-cell lymphoma, is increasing in incidence. However, many practitioners who treat patients with breast cancer are not aware of this disease.To assess how BIA-ALCL develops, its risk factors, diagnosis, and subsequent treatment and to disseminate information about this entity to the medical field.A literature review was performed in an academic medical setting. All review articles, case reports, original research articles, and any other articles relevant to BIA-ALCL were included. Data on BIA-ALCL, such as pathophysiology, patient demographics, presentation, diagnosis, treatment, and outcomes, were extracted. Particular focus was paid to age, time to onset, implant type, initial symptoms, treatment, and survival. The search was conducted in January 2017 for studies published in any year.After duplicates were excluded, 304 relevant articles were assessed, and 115 were included from the first documented case in August 1997 through January 2017. Thirty review articles, 44 case reports or series, 15 original research articles, and 26 “other” articles (eg, techniques, special topics, letters) were reviewed. A total of 93 cases have been reported in the literature, and with the addition of 2 unreported cases from the Penn State Health Milton S. Hershey Medical Center, 95 patients were included in this systematic review. Almost all documented BIA-ALCL cases have been associated with a textured device. The underlying mechanism is thought to be due to chronic inflammation from indolent infections, leading to malignant transformation of T cells that are anaplastic lymphoma kinase (ALK) negative and CD30 positive. The mean time to presentation is approximately 10 years after implant placement, with 55 of 83 (66%) patients initially seen with an isolated late-onset seroma and 7 of 83 (8%) with an isolated new breast mass. Ultrasonography with fluid aspiration can be used for diagnosis. Treatment must include removal of the implant and surrounding capsule. More advanced disease may require chemotherapy, radiotherapy, and lymph node dissection.Breast implant–associated anaplastic large cell lymphoma is a rare cancer in patients with breast implants but is increasing in incidence. It is important for all physicians involved in the care of patients with breast implants to be aware of this entity and be able to recognize initial symptoms.