The aim of this study is to describe the etiology, clinical presentation, radiographic diagnostics, and surgical treatment options of testicular anomalies and diseases in childhood, with a special emphasis on acute scrotum. Testicular anomalies and diseases include congenital anomalies, inflammations, cryptorchidism, tumors, and injuries of the testis and scrotal region. The most common cause of acute scrotal pain and swelling, also known as acute scrotum, in childhood are torsion of the testis and its appendages, inflammation of the testis or epididymis, testicular trauma, hernia/hydrocele, and neoplasia. Most of these conditions are nonemergent, but prompt differentiation and management are crucial, because testicular torsion results with permanent ischemic damage to the testicular tissues. Torsion of the appendix testis or epididymis is most common in prepubertal boys, and testicular torsion is more frequent in neonates and adolescents. Congenital anomalies of the testis and scrotal region are vary rare, but they take an important place in the differential diagnosis of acute scrotum and other similar conditions in pediatric surgery. Testicular tumors are uncommon in children, with a bimodal distribution of age-specific incidence rates and with two peaks at the ages 12 to 24 months and a second peak during puberty. Every solid scrotal mass should be considered malignant until proved otherwise. Although rare, testicular anomalies and diseases take an important place in the differential diagnosis and everyday work of each pediatrician and pediatric surgeon.