Background: Testicular adrenal rest tumors (TART) is there unspecified adrenocortical cells in the testis of patients with Congenital Adrenal Hyperplasia (CAH) and is similar to the hyperplastic adrenal glands and make testicular tumors that are known as Testicular adrenal rest tumor. These tumors are found frequently in adult man patients with CAH and are usually benign But due to tumor site in testicles, can lead to seminiferous tubule obstruction and gonadal dysfunction and infertility, therefore, early diagnosis and treatment of tumors is important. Due to the absence of exact statistics and studies to determine relative frequency Testicular adrenal rest tumor in patients with CAH and importance of early diagnosis of tumors in pediatric patients with CAH, This study was performed to determine relative frequency of testicular adrenal rest tumor in children with CAH.Methods: This study was a descriptive study that was performed in Al-azahra hospital in 1390. This study was performed on 44 patients with CAH under 20 years old. Height and weight and blood pressure was measured for each patient, also exact examination of testis and scrotum was done to diagnose of palpable mass. For all patients, ultrasound of testis was performed by an experienced radiologist. Assessment of patients was based on patient height and weight and serum level of 17 – hydroxyl progesterone. Finally, relative frequency of testicular adrenal rest tumor was evaluated in patient and its corelation with serum 17–hydroxy progesterone and dosage of medication and disease control rate and duration of disease was evaluated. The obtained data were analyzed by SPSS-18 software.Findings: The ultrasound exam results showed, TART was reported in 2 patients (4.5%) that in one of them, bilateral tumors was reported in the upper pole of both testis. and another tumor was visible in the Mediastinum of left testis. Both patients had type of salt loss. The average tumor size was 14 mm. None of the patients found palpable mass in the examination. Both patients with TART were in the age group 20-15 years old and patients with TART were under hormonal control and appropriate treatment. Serum levels of 17-hydroxy progesterone was less in patients with (TART) than patients without (TART). Any of 2 cases had impaired gonadal function.Conclusion: Testicular adrenal rest tumor (TART) occurs usually in severe forms of the disease (type of salt loss). Two cases with tumors (TART) in our study, were in the age group above 15 years old. It can be shown the correlation between duration of disease and prevalence of tumor. None of two cases found palpable mass in the examination. This can indicate that examination has not enough accuracy to detect tumor and is recommended for all patients, ultrasound be performed periodically.