Seven patients with xanthomas in the Achilles tendons and associated Type-II hyperbetalipoproteinemia are reported. Resection of the lesion in four patients was successful, and there was only one recurrence after six years. Two patients had painful xanthomas of their plantar fascia, similar to the involvement of the Achilles tendons. One had a resection of the plantar lesion. Of 173 cases of Achilles tendon xanthoma found in the English literature, 119 with adequate data are tabulated. Autosomal dominant inheritance and frequent association with extensor-tendon xanthomas of the hands and with tuberous xanthomas were noted. A strong tendency to recurrence was observed in surgically removed Achilles tendon lesions. Surgical intervention is carried out for cosmetic and symptomatic reasons.