Polymyositis (PM) and dermatomyositis (DM) are inflammatory myopathic diseases characterized by symmetric, proximal myopathy with or without a distinct cutaneous eruption. They have long been recognized to be associated with cancer. The common cancers associated with DM/PM include ovarian, lung, pancreatic, breast, and stomach cancer. PM/DM associated with hematological disorders and especially with acute myelocytic leukemia (AML) is extremely rare with very few cases being reported till date. In our review of literature we could find only seven such reported cases. We present here a case presenting with DM and diagnosed to be suffering from AML on admission. This happens to be the second reported case of DM and AML with no latent period between the two diseases.