Peripheral T-cell lymphoma (PTCL) is an aggressive tumor, rarely seen in pediatrics or adolescent and young adults (AYAs) so there is no upfront or relapsed standard of care. The authors describe a 16-year-old with PTCL, treated with chemotherapy and autologous stem cell transplant. Upon relapse, he received ifosfamide, carboplatin, etoposide, and radiation with durable remission of 4 years. Data in pediatric/AYA PCTL continue to lack an understanding of the biology and microenvironment, the differences to adult patients, and a lack of adequate therapy. Targeted therapy may improve outcomes for children and AYAs with refractory or relapsed PTCL.