Testicular adrenal rest tumors (TART) occur often as asymptomatic nodules in corticotropin- dependent lesions aberrant adrenal tissue in congenital adrenal hyperplasia (CAH) patients. The present manuscript is about an unusual case of a 16-year-old CAH patient due to 11β-hydroxylase deficiency. He underwent testicle biopsy because of testicle tumor suspicion and diagnosed with TART. © The Korean Urological Association, 2014.