A 17-year-old man was referred with a 6-month history of painless left inferior eyelid edema. Ophthalmic examination was normal except for the presence of a reddish conjunctiva in the left eye medial canthus. MRI demonstrated a 20-mm well-defined extraconical mass between the medial and inferior rectus muscle without destruction of the medial orbital wall. A low-grade myxofibrosarcoma was diagnosed on orbital biopsy. The tumor was locally excised, but the patient categorically refused left exenteration or conformal radiotherapy. He accepted to have annual clinical and radiologic checkup. To date, 2 years later, he has been followed up with no evidence of tumor recurrence. Orbital localization of myxofibrosarcoma is extremely rare, with only 2 cases reported in the literature. The differential diagnosis could be difficult: here, a fibromyxoid sarcoma and a myxoid variant of dedifferentiated liposarcoma have been especially discussed in terms of their histopathologic features. Risk of metastasis is related to the tumor grade. Management of these tumors associates surgery with orbital exenteration or globe-sparing approaches and postsurgical adjuvant radiation therapy.