Malignant histiocytosis is a fatal disease characterized by systemic proliferation of atypical, neoplastic and erythrophagocytic histiocytes. A Case of malignant histiocytosis with multiple nodular cutaneous involvement and eosinoptilia, in a 18 year-old male, was diagnosed on the third skin biopsy and reported. The first and second biopies shoved polymorphous, cellular infiltrates around the blood vessel and skin appendages in the lower dermis and diffuse infiltrates in the subcutaneous tissue. The infiltrates were composed of histiocytes, eosinophils, Iymphocytes and plasma cells without significant cellular atypism or abnormal mitoses. The third biopsy, which was performed 6 months later, when he readmitte4 due to aggrevation of the symptoms, showed monomorphous atypical histiocytic infiltrate with cellular atypism, abnormal mitoses, and erythrophagocytosis. Eosinophils, Iymphocytes and plasma cell infiltrates were markedly decreased. Brief review of the literatures with special reference to the association of cutaneous involvement and eosinophilia was made.