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Neurofibromatosis type 1 is the most frequent phacomytosis. Patients with neurofibromatosis type 1 (NF1) have a high risk to develop benign or malignant tumors of neurogenic or non-neurogenic origin. The association of (NF1) to low-grade fibromyxoid sarcoma (LGFMS) is very rare. These tumors rarely develop at the skull and have a high incidence of local recurrence and distant metastasis. We report a case of occipital LGFMS in a patient diagnosed with NF1that developed a local and metastatic recurrence.