'Aleukaemic leukaemia cutis' or acute leukaemia confined to the skin is extremely rare, although skin involvement with underlying leukaemia is well recognized, and is associated with a poor prognosis. We report a case of isolated acute myeloid leukaemia (AML) in the skin. A literature review shows this to be commonly misdiagnosed. Its recognition is important, because early diagnosis should lead to more appropriate chemotherapy, and a better prognosis. These patients probably require therapy directed specifically to the skin, as well as to other extramedullary sites, such as the central nervous system, to prevent early relapse.