Objective: Multicentric reticulohistiocytosis (MR) with systemic involvement is an extremely rare disease afflicting mainly adults. Method: We report a case of SR arising in a 3 year 7 month old boy with concomitant celiac disease. Results: Since 6-7 months, he developed several brown to dark-red skin nodules (up to 0.8 cm) located mainly on face and arms; current episode include hemorrhagic syndrome, severe pancytopenia and massive hepato-splenomegaly. Bone marrow aspirate ruled out acute leukemia and tezaurismoses; bone marrow biopsy and skin biopsy showed important infiltration with numerous large cells with abundant fine granular/ground glass eosinophilic cytoplasms in a reactive inflammatory stroma (lymphocytes, few plasma cells and few eosinophils); bone marrow cells were mononucleated, cutaneous cells were both mono&multinucleated; tumor cells immunophenotype was consistent with histiocytes (CD68+, S-100 protein + (faint), CD1a-, langerin-) both in bone marrow and skin biopsies; no hemophagocytosis was seen. MR with systemic involvement of skin, bone marrow, liver and spleen was diagnosed. Cyclophosphamide therapy was instituted with initial diminishing of splenomegaly; latter on, despite adding cyclosporine and etoposide, no further impact on pancytopenia and hepatosplenomegaly was recorded. Conclusion: Complete hematologic examination is mandatory for patients diagnosed with cutaneous reticulohistiocitosis, irrespective of their age, in order to identify MR and institute proper treatment.