Rhabdomyosarcoma (RMS) is a malignant tumor that arises from primitive mesenchymal cells. The most common site is the head and neck, accounting for 40%, followed by the urinary tract at 30%. Clinical suspicion, localization, age, imaging, immunohistochemistry, and molecular studies facilitate an accurate diagnosis and prompt treatment. The current clinical case demonstrates how the correlation of these clinical variables can hinder the progression of this neoplasm in a pediatric patient.