A 4-year-old boy presented with fatigue and was found to have severe kidney injury requiring haemodialysis. A renal ultrasound demonstrated bilateral nephromegaly with mild loss of corticomedullary differentiation but preserved echogenicity. He had a persistent isolated monocytosis. Renal biopsy revealed extensive infiltration by primary renal diffuse large B-cell lymphoma. He required haemodialysis for 18 days and received chemotherapy with cyclophosphamide, doxorubicin, vincristine, prednisone, rituximab and intrathecal methotrexate. He achieved remission with an estimated glomerular filtration rate of 50 mL/min/1.73 m2, and his kidneys returned to normal size. Nephromegaly due to renal-limited haematolymphoid disease is extremely rare, especially in children.
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