Background and Objective: Wilms’ tumor has been recognized as the most common primary malignancy of kidney at childhood, comprises 5-6% of tumors in this period, and manifests itself with various clinical symptoms. Since there have been no sufficient studies in this field in Iran, therefore, this study was conducted to investigate its histopathology and clinical symptoms. Materials and Methods: This study was carried out on existing data from 66 children with a diagnosis of Wilms’ tumor at children hospital during the years 1984-1999. In this regard, personal and disease-related characteristics of patients including age, gender, tumor stage, histopathology, and involved kidney were evaluated and SPSS software and Chi-square, t-test, ANOVA, and Mann- Whitney U test were used for data analysis. Results: The most common age of disease incidence was 2-4 years. In this regard, girl/boy ratio was 1.5. Meanwhile, the prevalence of an abdominal mass as the most common symptom was 83.3%. Left kidney was involved in 47% of cases and 55 of patients had a favorable histology. In addition, there was a significant correlation between site of kidney involvement and tumor histology (p<0.005). Conclusion: Considering the achieved advances in the diagnosis and treatment of Wilms’ tumor, prompt identification with regard to clinical symptoms can have a valuable role in its effective management.