The autopsy findings in a case of Bassen-Kornzweig's syndrome in which death was due to
cardiac failure are recorded. Interstitial myocardial fibrosis and long tract degeneration in the
spinal cord similar to that seen in fatal cases of Friedreich's ataxia were the chief cardiac and
nervous system findings. Excessive deposition of lipochrome pigment in intestinal and
cardiac muscle was a prominent feature. The implications of these findings and their
relationship to faulty lipid and vitamin absorption from the intestine are discussed.