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A 73 years old woman presented with 10 months history of inferior eyelid nodule, the biopsy revealed at the time a grade 2 leiomyosarcoma without metastasis, the excision was considered inappropriate and radiotherapy was performed. The patient was addressed for resistant ocular pain management grade 3 and persistence of the lesion despite all the treatments. The mass is now measuring 8 cm in maximal diameter. The MRI showed lesions that affect mainly the median extra orbital part. An embolization of the main artery that vascularise the lesion was performed, proceeded by wide exenteration. The patient died 4 months later of local recurrence in the orbital cavity and metastases. Leiomyosarcoma as a highly malignant character showed a furious resistance to chemotherapy, radiotherapy and embolization so wide radical exenteration from the beginning might give better chance at long term survival.