| Summary: | Pediatric soft tissue sarcomas comprise a small proportion of pediatric solid malignancies and may arise in numerous anatomic locations such as the head and neck, trunk, extremities, and the genitourinary tract. Here, we present a case of rhabdomyosarcoma (RMS) arising from an unusual location, the urachus. The 3-year-old patient initially presented with signs of small bowel obstruction. On diagnostic laparoscopy, an infraumbilical mass was discovered and removed by laparotomy. Analysis of the specimen confirmed embryonal RMS (ERMS). The patient was treated with a regimen of postoperative chemotherapy and radiation therapy. Although rare, prompt diagnostic evaluation and treatment of RMS may improve the long-term morbidity and mortality of these patients.
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