Acral myxoinflammatory fibroblastic sarcoma was first described in 1998. The tumor typically involves distal extremities; especially fingers and toes. It is usually located in subcutaneous tissue. The age range is broad (mean age 40). The size of tumor varies between 1-8 cm. Microscopically, myxoid and hyaline zones are characteristic. Inflammatory cells are abundant and there are large cells resembling Reed-Sternberg or ganglion cells and multivacuolated cells having lipoblast-like appearance.

We present 6 cases of acral myxoinflammatory fibroblastic sarcoma, locations were ankle, 1st toe and hand. The most presenting symptoms were swelling with or without pain and they have lasted for 3,5 months-4 years.

Immunohistochemical studies of our cases, revealed vimentin (5/5), CD68 (5/5), CD34 (1/3) positivity. Ki-67 proliferation indices varied between 2-10%. Five patients underwent tumor excision, while in one patient amputation had been performed.

This tumor is a low grade sarcoma but has the potential of recurrence and even metastasis, though it is rare. In one of our cases, recurrence was observed; in none of our cases metastasis has been determined.

It included many tumoral and non-tumoral lesions in differential diagnosis.