Macular amyloidosis is a major cause of skin pigmentation and a rare form of localized primary cutaneous amyloidosis in western countries, with a higher prevalence in Asia and South America. The etiopathogenesis of macular amyloidosis has not yet been fully clarified; a number of risk factors are involved, among them chronic friction in particular. A 54-year-old patient with macular amyloidosis is presented. The diagnosis of macular amyloidosis was based on history data on long-term persistence of the disorder, localized pruritus and constant scratching urge, grayish-brown pigmentation over the scapula, and detection of amyloid in histologic slides. Three-month tretinoin therapy resulted in pruritus alleviation, with no change in the appearance of hyperpigmentation. The exact incidence of macular amyloidosis in Croatia is not known, however, the issue appears to be underestimated or neglected in dermatology routine.