Amyloidosis is a rare disease without known etiology. Though microscopically there is no distinction between its various types, clinically amyloidosis may be divided into four groups: primary systemic amyloidosis unassociated with other underlying disease; secondary amyloidosis associated with chronic debilitative diseases; amyloidosis associated with multiple myeloma; and localized amyloidosis involving a single area or organ. In recent years, the sharp decline in incidence of osteomyelitis and tuberculosis has shifted the interest in amyloidosis from the secondary to the primary form. Fewer than 200 cases of primary amyloidosis have been reported, most of them in the past 50 years.1 The following case of primary systemic amyloidosis was of particular interest because the patient's presenting complaints were jaundice and hemorrhage, unusual symptoms in amyloidosis. Because the diagnosis of extrahepatic biliary obstruction could not be excluded, exploratory celiotomy was necessary to establish the diagnosis of primary systemic amyloidosis. Report of a Case