Acquired clonal chromosome X aberrations, whether numerical or structural, as the sole chromosomal anomaly in acute myeloid leukemia (AML) are very uncommon. The scarcity of nonconstitutional chromosome X aberrations detected in AML has prevented any meaningful evaluation of their prognostic significance. In this report, we describe the case of a patient with AML who had the unusual acquired karyotype of 45,X/-X,46,X,i(X)(q10) on presentation and 45,X,-X,add(19)(p13.3) on relapse. We also briefly review the literature on acquired numerical and structural chromosome X aberrations as the sole chromosomal anomaly in AML. This case adds to the sparse body of literature regarding chromosome X aberrations in AML. More case reports are needed to further elucidate the importance of such aberrations.