Objective: Low grade fibromyxoid sarcoma (LGFMS) is a specific type of fibrosarcoma with deceptively banal appearance and malignant behavior. Method: A 5 year retrospective study revealed 10 LGFMS cases. Morphological and immunohistochemical analysis was performed. Follow-up information was obtained for 5 cases. Results: All tumors occurred in adults (mean 40 year). They developed in neck (1), extremities (6) and trunk (3). Beside classical morphology of LGFMS following features were observed: necrosis (3), rosettes (3), areas of increased cellularity (5), foci of epithelioid cells (5), marked nuclear pleomorphism (1), prominent myxoid change (3), invasive border (6), bone formation (1), focal retiform pattern (1) and sclerosing epithelioid fibrosarcoma-like areas (1). Immunoprofile was the follow: vimentin (100 %), EMA (30 %), CD34 (10 % focally), S100 (0), SMA(0), desmin (0), AE1/AE3 (0). Two patients were without evidence of disease, one developed lung metastasis after 1 year. Two patients developed secondary tumours in 6 and 12 years after initial diagnosis. There were differences in the location and histologic features compared with primary ones. Secondary tumors were less cellular, without necrosis or epithelioid cells. Behavior of secondary tumors did not fit to the classical concept of tumor progression that implies increase of malignancy. Conclusion: