Low grade fibromyxoid sarcoma is a recently recognized, uncommon soft tissue neoplasm. It has a tendency to develop in deep soft tissue of young adults and a possibility of local recurrence or distant metastasis. Diagnostic criteria have not been well defined and this tumor has not been accepted as a distinct entity. Histologically, it is characterized by the presence of bland spindle cells with mainly whorled pattern of growth, set in alternating areas with a myxoid or fibrous stroma. Careful consideration of the morphological and immunohistochemical features of this tumor permit a positive diagnosis of low grade fibromyxoid sarcoma and allow its distinction from a number of other benign and malignant soft tissue neoplasms. We experienced a low grade fibromyxoid sarcoma in chest wall and report this case with a review of the literature.