Dermatomyositis (DM) is a dermatomyopathy with a broad spectrum of features, defined by Bohan et al as an objective set of clinical and laboratory criteria. Dermatomyositis has been associated with extracutaneous neoplasias, especially with ovarian carcinoma. The term “punctate keratoderma” comprises a group of diseases with different etiologies characterized by small hyperkeratotic lesions usually scattered in palms and soles. Punctate porokeratotic keratoderma (PPK) is a type of punctate keratoderma mainly defined by its peculiar histological features, including the presence of cornoid lamella similar to the one observed in porokeratosis. Punctate porokeratotic keratoderma has been considered a sporadic disease although some rare paraneoplastic cases have been published. We report for the first time a case of dermatomyositis with typical features of punctate porokeratotic keratoderma, both as paraneoplastic presentation of ovarian carcinoma. Its paraneoplastic nature is clearly demonstrated by the clinical improvement observed following tumor excision.