SummaryProgressive nodular histiocytosis is an extremely rare skin disease is clinically characterized by the coincidence of two distinct lesions, namely, superficial xanthomatous papules up to 5 mm and deep nodules and tumors 1–3 cm. Histologically the nodules represent spindle cell xanthogranulomas. We report a 24‐year‐old women with these findings. The distinction from other non‐Langerhans cell histiocytoses, in particular multiple juvenile xanthogranulomas, which may be more likely to show spontaneous remission, is somewhat unclear; patients with progressive nodular histiocytosis usually follow a serious and disfiguring clinical course.