BACKGROUND Langerhans cell histiocytosis (LCH), also called histiocytosis X, belongs to a group of rare neoplasms and is a clonal pathology characterized by infiltration of Langerhans cells. The pathology can occur with the involvement of only 1 organ, more frequently the bone or with multi-visceral involvement, and patients frequently receive a delayed diagnosis and empirical treatments. CASE REPORT We report a case of LCH in a 60-year-old woman who presented atypical symptoms, imaging findings of lung and liver involvement. Imaging showed increased liver volume and subverted structure by multiple nodular formations. For the differential diagnosis with other neoplastic liver diseases, the patient underwent liver biopsy, with microscopic typical findings of the disease and positive immunohistochemical markers. CONCLUSIONS Liver involvement in LCH is rare and the differential diagnosis with neoplastic pathology may pose a challenge for the clinician and radiologist, also due to the possible association between LCH and malignant tumors.