Testicular relapse of acute myeloid leukemia without bone marrow involvement is a rare event. We describe a case of an 18‐year‐old male who had an isolated testicular relapse 86 months (7.2 years) from original diagnosis. He was treated with surgery only, without adjuvant therapy. The patient then developed central nervous system involvement 9 months later. Fluorescence in situ hybridization and immunohistochemistry were used to establish the diagnosis of a relapse rather than a new leukemic process. He was treated with intrathecal chemotherapy and systemic reinduction, followed by a stem cell transplant. This patient had a 7.2‐year period between original diagnosis and the testicular relapse of acute myeloid leukemia. Pediatr Blood Cancer. 2010;55:1231–1233. © 2010 Wiley‐Liss, Inc.