Benign cephalic histiocytosis (BCH) is a rare, benign, non-Langerhans cell histiocytosis seen in infants and young children. It is characterized by a self healing eruption of multiple yellow to red-brown papules on the head and neck, occasionally spreading to the trunk and extremities. Recent studies suggested that BCH showed overlapping clinical, histologic, and ultrastructural features with juvenile xanthogranuloma (JXG) and generalized eruptive histiocytoma, which indicated they may belong to a one wide spectrum of non-Langerhans histiocytosis. A 12-month-old boy presented with scattered erythematous-yellow papules on the face which had appeared 4 months ago. Mucosa, palms, and soles were spared. He was otherwise healthy and developmentally normal. BCH, JXG and verruca plana were considered as differential diagnoses. Histopathological examination showed the proliferation of epithelioid histiocytic cells with amphophilic cytoplasm and prominent nucleoli in the upper and mid-reticular dermis. Immunohistochemically, the histiocytes were positive for CD68 while lacking expression of S-100, which helps to confirm the diagnosis as benign cephalic histiocytosis. As a few BCH cases have shown transformation into JXG involving ocular sites, he was recommended the ophthalmologic examination which revealed no other abnormalities. As there have been only four cases published in Korean literature; herein we decide to report a rare case of BCH.