We report successful percutaneous nephrolithotomy (PCNL) in an 8-week-old, 4.12 kg infant with a combined stone burden of > 2 cm in a solitary kidney. The patient was born with thoracolumbar myelomeningocele and had developed recurrent urinary tract infections. Her size precluded retrograde intrarenal surgery and shockwave lithotripsy would be unlikely to clear the stone burden. Stone analysis revealed hydroxyapatite and carbonate apatite stones, and metabolic work up revealed hypercalciuria for which chlorothiazide was started. To our knowledge, this is the youngest patient to undergo PCNL reported in the literature.