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Teratoid Wilms Tumor (TWT) is a rare renal malignancy that can masquerade as a cystic dysplastic kidney in young children. We report a 3-month-old child with a prenatally detected left renal cystic lesion initially diagnosed as multicystic dysplastic kidney (MCDK). Atypical imaging findings prompted further evaluation, revealing TWT. Histopathology confirmed heterologous elements and focal Wilms tumor components. This case underscores the need for vigilance in cystic renal lesions, as early recognition of malignancy alters management and improves outcomes.