We present a rare case of multisite xanthomatosis occurring in a 38-year-old normocholesterolemic man without a familial history. More commonly, these conditions are associated with familial hypercholesterolemia and cerebrotendinous xanthomatosis. Tendon xanthomas should prompt clinicians to perform a thorough investigation of the patient's metabolic panel and family history. Rarely, these conditions occur in patients without demonstrable dysmetabolic state. The characteristic MR imaging findings are presented in conjunction with review of the literature.