We reported on a rare case of recurred macrodystrophia lipomatosa of the foot, and reviewed the literature. A 62-year-old male patient presented with right foot second toe pain; preoperative magnetic resonance imaging and radiograph examination was performed. After surgery the biopsy confirmed the diagnosis. American Orthopaedic Foot and Ankle Society score was checked before and after surgery. Wide excision of the affected area including ray amputation is an effective way to prevent recurrence and relieve the pain after surgery. The 2nd toe ray amputation was performed in the treatment of recurred macrodystrophia lipomatosa of the foot, and is thought to be an effective way to relieve pain and prevent recurrence. After minimally invasive surgery with complete excision surgery, additional data on recurrence and pain relief rate are needed.