Congenital malaria, an uncommon disease in the United States, may result in serious morbidity when not promptly diagnosed. All cases of congenital malaria known to have been seen in the United States since 1950 are reviewed and the most recent case is presented to illustrate the salient features of this disease. Congenital malaria may remain undiagnosed for a prolonged period unless considered in the differential diagnosis of fever, anemia, and splenomegaly in an infant less than 4 months of age whose mother's travel history is unknown. This circumstance often results in the performance of unnecessary procedures, ineffective treatments, and potentially significant morbidity and expense.