Malignant rhabdomyosarcoma develops from early muscle cells that develop abnormalities that are usually caused by genetic variants. The currently known genetic variants can explain only a very small number of cases, and the majority of cases remain without a clear pathogenesis. The underlying etiology lies in the fact that rhabdomyosarcoma originates from rhabdomyoblasts, which may develop into rhabdomyosarcoma when there is an abnormality in the division and proliferation of rhabdomyoblasts. Rhabdomyosarcoma is the most common type of malignant tumor among children’s soft tissue sarcomas and can occur in various parts of the body, commonly in the head and neck, limbs, and genitourinary organs. It can be classified as embryonal rhabdomyosarcoma, vesicular rhabdomyosarcoma, pleomorphic or mesenchymal. Certain genes or chromosomes are known to be involved in the development of rhabdomyosarcoma, such as heterozygous deletion of chromosome 11 in some embryonal rhabdomyosarcomas, chromosomal translocations in some adenomatous rhabdomyosarcomas, e.g., translocations of chromosomes 2 and 13 between bands 5 in long arm 3 and band 4 in long arm 1, and translocations of chromosome 1 and chromosome 13 to form the fusion genes PAX3 and PAX7, and also some rhabdomyosarcomas. Some rhabdomyosarcomas are also associated with mutations in the TP53 gene, which predispose to genetic disorders such as congenital syndromes that are associated with an increased risk of rhabdomyosarcoma, including Liverauményi cancer susceptibility syndrome, other type I neurofibromatosis, and Behvi syndrome. However, these genetic or congenital risks are very rare and do not imply a certainty of developing rhabdomyosarcoma.