Atypical Teratoid Rhabdoid Tumors (AT/RTs) often affect children under the age of 3 and are the most common malignant CNS tumors in children younger than 6 months. It is very rare to see these tumors in patients older than 6 years of age. We discuss the case of a 14 year old male with AT/RT of the right insula. He had a prior diagnosis of Dysembryoplastic Neuroectodermal Tumor (DNET) at the age of 10, after two years of intermittent headaches, nausea, and seizures, which was treated with conformal radiation and chemotherapy for a year. Following the diagnosis of AT/RT, he underwent radiotherapy, multiple lines of chemotherapy, and two additional debulking surgeries of the left temporal lobe due to continuing progression. He was then treated with Alisertib (an Aurora-A kinase inhibitor) with good response on sequential MRIs after the first three cycles. He progressed after nine cycles of Alisertib and required further debulking surgery. Six years after his AT/RT diagnosis (and 10 years after his DNET diagnosis), the patient expired at the age of 20 due to ongoing progression. To our knowledge, this is only the second reported case of Alisertib use in a non-pediatric AT/RT case. We also performed a literature review of all reported cases of AT/RT in adults between the years 2000 – 2017 and discuss treatment options, patient demographics, and survival.