We describe a four-week-old male infant with bilateral renal parenchymal malakoplakia who presented with low grade fever, convulsions and lethargy. The patient had profound anemia, hepatosplenomegaly and bilateral nephromegaly with reduced renal function. Both blood and urine cultures grew Escherichia coli, and antibiotic therapy was started. A kidney biopsy obtained on the 20th hospital day confirmed the diagnosis of renal parenchymal malakoplakia. Following treatment with an intravenous methylprednisolone pulse therapy, the infant made significant clinical improvement. He has grown and developed normally in the three years following this episode. We suggest that the steroid therapy was useful in ameliorating renal parenchymal malakoplakia in a patient without an underlying systemic disease. This report describes the youngest patient to have malakoplakia.