One case of acral myxoinflammatory fibroblastic sarcoma is presented. Most of the soft tissue tumefactions of the distal extremities are benign. Sarcomas are rare and usually high grade. Acral myxoinflammatory fibroblastic sarcoma is a unique low-grade tumor described by Kindblom in 1998. This neoplasm has a propensity for digits, presents a low rate of metastasis and is characterized by a high rate of local multiple and aggressive recurrence. The clinical presentation, the frequent association with tenosynovitis and the extensive inflammatory infiltrate can lead to misdiagnosis by both the surgeon and the pathologist. Correct and early diagnosis depends on a good knowledge of histological and immunohistochemical findings. It is important to avoid recurrences recurring to an immediate wide excision of the tumor.