Introduction: Teratomas are derived from embryonic tissues that are typically found in the gonadal and sacrococcygeal regions of adults and children. Primary teratomas in the retroperitoneum are very rare in infant and primary adrenal teratomas are extremely rare. Early diagnosis and surgical resection are important for effective treatment. Case report: We report here the case of a histologically unusual adrenal teratomas detected on computed tomography during the workup of abdominal distension 3-mounth-old male infant. The evaluation and treatment of this condition and a review of the literature are included in this paper. © AVICENA 2013.