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Wilms tumor (WT) is one of the most common solid tumors in children, accounting for 5–10% of all pediatric malignancies. This paper describes four cases of WT, each of which demonstrates particular characteristic.Case 1: A 3-year-old male diagnosed with a WT uniquely located in the isthmus of a horseshoe kidney. He underwent bilateral double J catheter insertions, tumor resection with partial isthmus nephrectomy, and lymphadenectomy.Case 2: A 3-year-old male presented with hematuria. Abdominal computed tomography (CT) revealed a left renal pelvis mass suspicious for urothelial neoplasia. The patient underwent total left nephroureterectomy with partial cystectomy.Case 3: A female patient with a history of right-sided WT at age 2, was readmitted as a 14 year old with a large left-sided renal mass and smaller bilateral pulmonary masses. She underwent tumor resection which revealed renal cell carcinoma.Case 4: A 2-year-old male patient underwent multiple surgeries after being diagnosed with bilateral WT. Later, the patient was found to have multiple hepatic metastases and underwent right hepatectomy using the associating liver partition and portal vein ligation for staged hepatectomy (ALPPS) procedure.