Objective: Langerhans cell histiocytosis is a rare disease histologically characterized by the proliferation of Langerhans cells. Method: A 65-year-old male came to our attention presenting with erythematous-squamous lesions all over the body, axillary and inguinal adenopathies and hepatosplenomegaly. We have taken a cutaneous and an axillary lymph node biopsy. Results: Microscopic examination of the cutaneous fragment revealed the presence of a dermal infiltrate of large cells with an eosinophilic cytoplasm and vesicular, nucleolated nuclei with an irregular nuclear margin, raising suspicion of a cutaneous lymphoma. Immunohistochemical stains for CD45, CD20, CD79a, CD3, CD5, CD15, CD30, and Bcl-2 were negative, and the previous diagnosis was not confirmed. The microscopic examination of the axillary lymph nodes showed foci of extramedullary hematopoiesis, raising suspicion of a lymphoproliferative process, but the immunohistochemical stains and the paraclinical data didn't confirm it. Based on the intense positive reaction for CD68, S100 and Ki-67 showed by the tumoral cells from the prelevated tissues, correlated with the clinical and paraclinical findings, a diagnosis of Langerhans cell histiocytosis was established. Conclusion: The particularity of our case consists in the presence of extramedullary hematopoiesis in the lymph nodes of a patient diagnosed with Langerhans cell histiocytosis.