The authors describe a rare case of amyloidosis in a female patient suffering from periodic disease (PD) for 18 years without any clinico-laboratory signs of renal impairment but with marked clinical, (malabsorption, cachexia), endoscopic, x-ray and other manifestations of gastrointestinal amyloidosis. This case is of interest since patients suffering from amyloidosis due to PB develop malabsorption very rarely, namely in 2-3% of cases. As a rule, it develops in patients with pronounced chronic renal failure on hemodialysis or with a history of kidney transplantation. In this particular case, the patient demonstrated selective marked damage to the gastrointestinal tract, with the kidneys remaining practically intact. A possibility of the indicated variety of amyloidosis should be considered in specification of the genesis of persistent diarrhea in PB patients.