Rhabdomyosarcoma is the most common primary malignant connective and soft tissue neoplasm in the paediatric population. It arises from undifferentiated mesenchymal cells that have the potential to differentiate into skeletal striated muscle. It can develop almost anywhere on the body, but it is characterized by a predilection to the tissues of the head and neck, as well as the genitourinary system. Incidental diagnoses of testicular rhabdomyosarcoma in the asymptomatic disease stage are extremely rare. Our article is a case report of a 14-year-old boy with symptoms of acute scrotal syndrome (ASS), which occurred a few days after the injury of this area. Due to the suspicion of testicular torsion or testicular appendix torsion, an urgent scrotal revision was performed. It revealed the presence of a heterogeneous tumour rooted in the right testicular fascies. The result of the histopathological examination allowed the diagnosis of highrisk rhabdomyosarcoma. The performed additional diagnostic tests did not reveal any dissemination of the neoplastic process. Accidental diagnosis of the malignant neoplasm at an asymptomatic disease stage allowed early treatment and increased the chances for a cure. Although neoplasms are rare causes of acute scrotal syndrome, the described case indicates that special oncological awareness should be preserved in every patient with symptoms of acute scrotum.