Xanthoma is a localized collection of tissue histocytes containing lipid. The majority of tendinous xanthomas probably occurs in the setting of hypercholesterolemia especially in bilateral Achilles tendon xanthomas. Xanthoma of the Achilles tendon is a rather rare, interesting orthopaedic condition that has important ramifications in internal medicine and dermatology because the lesion is associated with a specific disturbance of lipid metabolism. We experienced one case of normolipidemic and symptomatic Achilles tendon xanthoma. Surgical intervention was carried out for cosmetic and symptomatic reasons, the patient undergoing total resection and a reconstruction of the Achilles tendon by the combinedV-Y muscle flap and modified Lindholm technique.