Introduction/Aim. Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in pediatric population and adolescents. Limited data is available on the characteristics of RMS in Iraqi pediatric patients. The aim of the study was to examine the clinical and histological aspects of RMS in Iraqi children, with a focus on their response to treatment, prognosis, and survival. Methods. A retrospective cohort study was conducted at the Oncology Unit of Children's Welfare Teaching Hospital, Medical City, Baghdad, Iraq and included patients who were newly diagnosed with RMS and received treatment during the period between January 1, 2015, and December 31, 2019. The patients were followed up from the time of diagnosis until October 1, 2020. Results. A total of 59 patients were included with a median age of 3.5 years ranging between 1-12 years, with a male-to-female ratio of 3.2:1. The most frequent clinical presentation was urine retention in 15 patients (25.4%), followed by proptosis in 14 (23.7%) patients. However, the main sites of involvement were the head and neck in 23 (39%) patients, followed by the trunk and the genitourinary tract observed in 17 (28.8%) and 15 (25.4%) patients, respectively. The alveolar type, found in 11 (18.7%) patients, was the most prevalent histological variety, followed by the embryonal type reported in 42 (71.3%) patients, whereas six patients (9.7%) had other varieties. Of the 49 patients included in the prognosis assessment, 18 patients (36.7%) had a complete response, 20 patients (40.8%) died, and 11 patients abandoned treatment. The average duration of disease recurrence was 21.3 months, with a 7% recurrence rate. The overall survival rate was 36.7%, with a mean survival duration of 14.1 months. Conclusions. Pediatric RMS in Iraq is primarily prevalent in males. There is a significant delay in disease diagnosis from the onset of the symptoms. A high rate of advanced-stage disease may relate to patients reluctant to discontinue treatment. The low disease-free survival rate is due to impediments that hindered the effectiveness of therapy.