A 66 year-old woman was referred in 1981 because of renal insufficiency and pronounced nephrocalcinosis. The urinary oxalate excretion was elevated. Secondary hyperoxaluria was excluded. End-stage renal disease necessitated hemodialysis from late in 1982 up to her death in 1986, at the age of 71 years. During the course of the disease, an aggressive arthropathy developed in the fingers. Classical signs of oxalosis were found: deposits of calcium oxalate crystals in bone tissue, the pancreas, myocardium, subcutaneous tissue and especially in the kidneys. This rare case documents the possible occurrence of late clinical presentation and long survival in primary oxalosis.