We report a case of a 3-month old male infant, born to a mother with a known history of systemic lupus erythematosus (SLE). The infant initially presented with petechiae, anemia, and thrombocytopenia. His evaluation revealed antinuclear antibody (ANA) titer of 1 : 160, negative anti-SS-A/SS-B antibody, positive anti-Smith antibody, elevated anti-dsDNA titer, and a slightly low C4 level. His subsequent development of hematuria with nephrotic grade proteinuria fulfilled criteria for a diagnosis of SLE. His condition improved with corticosteroids, mycophenolate mofetil and low-dose aspirin. At 18 months of age, he is clinically well, off all immunosuppression with normal growth parameters and no detectable autoantibodies.