We report the case of a 20-year-old woman with one-year history of asymptomatic pink papules on the abdomen, with central atrophy. Fever and symptoms suggesting involvement of other organs were absent. Histological examination revealed wedge-shaped area of cutaneous ischemia extending into the deep dermis with superficial and deep perivascular lymphocytic infiltrate. On this basis, we diagnosed malignant atrophic papulosis. Laboratory tests and instrumental investigation did not reveal any systemic involvement. The rarity of this disease makes early diagnosis challenging, even if clinical and histological patterns of the skin lesions are peculiar.