Although rhabdomyosarcoma is the most frequent soft tissue tumor in children, there are extremely few reports of this tumor arising from the urachus. The authors describe another case in a 6-year-old female associated with constipation and a painless suprapubic mass. The specimen had the pathological criteria used to define urachal sarcoma (cytological, histological, and immunohistochemical findings) and urachal remnants were not observed. After complete resection of the primary tumor the patient was treated with chemotherapy (ifosfamide, vincristine, and dactinomycin) and remains alive at 4 years' follow-up with no signs of recurrence. Nowadays the management of children with urachal rhabdomyosarcoma usually includes surgery and chemotherapy. Multimodal therapy and surgery are constantly evolving and have significantly improved overall survival of these patients especially in R0 resected patients.
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