Juvenile xanthogranuloma is a benign, self-limiting tumor that arises from histiocyte proliferation of unclear aetiology in children. Cutaneous manifestation is most common, and systemic involvement rarely occurs. Early diagnosis, on the other hand, is necessary for further evaluation in order to avoid disease complications later on. A four-month-old baby boy was referred to a dermatology clinic by the paediatric team because he had multiple skin lesions. Multiple well-defined borders, soft, non-fluctuant yellow and reddish papules or nodules ranging in size from 2.0 to 4.0 cm, have been seen over the scalp, face, chest, upper back, and upper limbs since birth. The examination of the eyes, heart, lungs, and abdomen is unremarkable. The results of additional imaging to test for systemic involvement were negative. A skin biopsy shows dense dermal infiltration by sheets of mononuclear histiocytes, from polygonal to spindle-shaped and plump, possessed of abundant pale eosinophilic cytoplasm with indistinct cytoplasmic borders. These lesion cells are diffusely positive for CD68 and negative for CD1a and S100. The features are consistent with juvenile xanthogranuloma. Following an 11-month follow-up, the skin lesion was much flatter and softer than before. Although systemic involvement with cutaneous juvenile xanthogranuloma is uncommon, a thorough examination is required to avoid conditions that are associated with high morbidity and mortality, such as hepatic failure, progressive central nervous system disease, and myeloid leukaemia.