Testicular torsion is a commonly encountered medical emergency in children. A 10-year-old boy with diagnostically confirmed leukemia presented with new onset testis swelling. Scrotal ultrasound showed absent blood flow on the left, consistent with acute testicular torsion. The patient underwent left orchiectomy due to the testis being unsalvageable. Later pathology confirmed lymphoblastic infiltrates. A malignancy of the testicles is rarely associated with torsion and, in the setting of leukemia, suggests widespread disease. Due to the risk of scrotal violation, an inguinal approach is preferable for surgical exploration of the testicles in patients with a history of leukemia.