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This article highlights the case of a 74-year-old Caucasian female who presented with jaundice, clay-colored stools, diarrhea, and fatigue of 3 months' duration, accompanied by a weight loss of 6.8 kg. The results of initial investigations were interpreted as primary sclerosing cholangitis, but futher investigation revealed lytic and and blastic bone lesions. A sacral bone biopsy established the diagnosis of systemic mastocytosis.