The non-Langerhans cell histiocytosis (non-LCH) is a group of diseases characterized by cutaneous involvement in the neonatal period. The non-LCH affects less than 1 in 200,000 children born each year. A definitive diagnosis is important for the treatment of the disease. Therefore, skin biopsies should be performed in neonates with cutaneous lesions as early as possible. Only in the presence of cutaneous involvement without systemic involvement, it does not require any treatment, because the lesions can mostly self-heal. Here, we present a neonate diagnosed with non-LCH following a skin biopsy.