Background Tendon xanthomatosis is often associated with familial hypercholesterolemia, but it can also occur in other medical conditions. The Achilles tendon is the most common site of tendon xanthomas. Reconstruction of large defects after the xanthoma excision, can be challenging. Methods We propose a novel technique for Achilles tendon reconstruction with the use of an ipsilateral autologous semitendinosus tendon graft. The technique consists of six steps. Results This procedure has a low rate of complications and provides results that are at least comparable with those reported with other surgical approaches.