BACKGROUND Desmoid fibromatosis is a rare benign neoplasm arising from musculoaponeurotic structures with the potential to infiltrate local tissues and recur locally. While the commonly affected regions are the shoulder, chest wall and back, thigh, head and neck, localization in the foot is rare and only a few cases have been reported. Deep-seated lesions of foot tend to spread along the fascia and nerve or tendon sheaths. METHODS We present a case of a 41-year-old female with a 3 years history of a slowly enlarging painful mass in the right foot. METHODS Desmoid fibromatosis was diagnosed based on clinical manifestations, imaging findings and pathological examinations. METHODS The patient was treated by local incomplete resection. RESULTS The patient recovered well postoperatively and was well followed up at our outpatient department with no evidence of recurrence during 16 months of follow-up after local excision. CONCLUSIONS Surgical excision is recommended for symptomatic lesions located in the foot. However, it is difficult to excise thoroughly and periodic follow-up is needed to monitor for recurrence. Further study is warranted to determine whether patients benefit more from function preservation instead of complete resection.