Angioimmunoblastic T-cell lymphoma (AITL) is an uncommon subtype of Peripheral T-cell lymphomas (PTCLs) in children with less than 20 cases reported in the literature. In this paper, we present a case of AITL in a three years old male with Ataxia-Telangiectasia which regressed spontaneously without any therapy. A comprehensive review of the literature regarding treatment and outcome of pediatric patients with AITL has been also discussed in this article.