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Acute scrotum syndrome in children is characterized by acute scrotal pain, accompanied by inflammatory signs. The most common causes are torsion of testicular appendages, torsion of the spermatic chord and epididymitis/orchitis. In this review, we describe the clinical features, diagnostic methods and treatment of these pathologies. We also highlight the importance of early differential diagnosis because timely treatment of the spermatic chord torsion reduce the risk of necrosis in the affected testes.