Aplastic anemia is a rare but serious complication of systemic lupus erythematosus (SLE) with an often dramatic and unanticipated onset. The peripheral destruction of formed blood elements, which frequently accompanies the syndrome, may obscure or delay the diagnosis of bone marrow suppression, and the number of published cases may be an underestimate of the actual incidence of the disease. Furthermore, the disease course may differ significantly from other forms of acquired aplastic anemia and seems to carry a more favorable prognosis once effectively diagnosed and treated. In addition, aplastic anemia may precede other manifestations of SLE. Therefore, the possibility of bone marrow aplasia should be excluded in all SLE patients with severe pancytopenia, and conversely, the diagnosis of SLE should be explored in cases of aplastic anemia.Two patients with aplastic anemia in SLE, one with aplastic anemia preceding the onset of SLE, are described along with 15 cases reviewed from the English language literature. The presentation, prognosis, treatment, and pathogenesis of aplastic anemia complicating SLE are discussed. Recognition that cytopenias, especially pancytopenia, may occur on the basis of inhibited myelopoesis rather than peripheral destruction as either a harbinger of SLE or as a manifestation of disease flare is important. This knowledge will prompt the astute clinician to obtain screening antinuclear antibodies in the setting of otherwise unexplained bone marrow acellularity or, given the prognosis of SLE associated aplastic anemia, give early consideration to more aggressive immunosuppression.