The onset of a dermatomyositis-like rash and persistent skin ulcers during long-term treatment with hydroxyurea is a very rare event that has not been fully described in the literature. The fact that these lesions have a typical clinical presentation and course, and that complete resolution can only be achieved by immediate withdrawal of the drug, means that dermatologists should be aware of this condition in order to avoid a delay in diagnosis. We present the case of a 76-year-old woman who developed a dermatomyositis-like eruption associated with chronic ulcers on the lower limbs during long-term treatment with hydroxyurea.