Bullous pyoderma gangrenosum begins as a bulla, nodule or nonulcerated erythematous plaque that blisters or ulcerates to form a superficial ulcer surrounded by a hemorrhagic, bullous border, which is surrounded by a blue-gray halo. Bullous pyoderma gangrenosum is most commonly associated with hematologic malignancies, specifically, acute myelogenous leukemia (AML). We report a patient whose initial presentation with bullous pyoderma gangrenosum prompted the appropriate diagnostic evaluation and confirmation of AML, which was ultimately fatal. We emphasize that a thorough hematologic investigation, including bone marrow biopsy, should be performed in all patients who present with lesions clinically suggestive of bullous pyoderma gangrenosum because the skin lesion may be the only indicator of the underlying hematologic disorder.