Renal-cell carcinoma, clear-cell sarcoma, (congenital) mesoblastic nephroma, rhabdoid tumour, and renal medullary carcinoma form a heterogeneous group of childhood renal malignancies known as non-Wilms' tumours. Progress has been slow in improving the management of these tumours to decrease morbidity and increase survival. However, greater cooperation between national and international centres should engender specialisation, and an increased knowledge of the molecular biology of these tumours will inevitably lead to substantial progress over the next decade. This review is the second of two parts: the first part provided an updated review of the clinical presentation, imaging, and pathology of non-Wilms' tumours and this second part provides an updated review of the treatment of these tumours.