| Summary: | Thromboembolism is infrequent in children, nonetheless, whenever malignancy is present, there is an increased risk, around 7% to 25%. Rhabdomyosarcomas are typically asymptomatic masses, but patients can become symptomatic due to mass overgrowth and compression of surrounding structures, therefore presentation might be as a thromboembolic event. Sarcomas have recognized risk factors including large tumour masses, intensive chemotherapy, radiation therapy, extensive surgery and associated limited mobility. Whenever a thromboembolic event presents without a reliable aetiology and with no response to conventional treatment, an underlying malignancy should be excluded. In this context, we present a six-year-old boy who presented with a persistent symptomatic deep venous thrombosis of the inferior limb, despite anticoagulation and, in the subsequent investigation, a retroperitoneal rhabdomyosarcoma was diagnosed. In the light of this case, a brief review of the literature review is presented.
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