Abstract We report a case of clear cell sarcoma in the third metatarsus of the right foot. This type of tumor is very rare and scantily reported in literature. A 42-year-old Caucasian male presented with a nodular ulcerated mass on the dorsal side of the left foot. X-rays demonstrated a nodular solid lesion which dislodged the third metatarsus. A biopsy revealed a neoplastic proliferation with a sarcoma clear cell profile; because of the aggressive nature of this type of neoplasm, we performed a trans-tibial amputation according to Bugess to achieve a better functionality for the patient. The present study underlines clinical, morphological, as well as imaging and therapeutic aspects of a rare neoplasm such as clear cell sarcoma. The location site is also quite unusual - the metatarsus of the foot. The histological and immunohistochemical data were suggestive of the diagnosis of clear cell sarcoma of metatarsus. After MRI and a bone scan, the surgical treatment suggested the extension over the forefoot and the ankle and therefore a trans-tibial amputation was made.