A 4-month-old infant presented with an enlarging large vascular presternal swelling noticed for the past 2 months. Clinical examination revealed a left renal mass in this otherwise asymptomatic child. She underwent left nephroureterectomy and excision of the skin metastasis following a course of chemotherapy. Preoperative cytology and postoperative histopathological examination confirmed Wilms' tumor with a single skin metastasis. We report this case for its rarity.