Males are affected with Bassen-Kornzweig syndrome with greater frequency (70%) than girls, which is uncharacteristic in most autosomal recessive conditions.

Bassen-Kornzweig syndrome is inherited as an autosomal recessive disorder, which means that parents of affected individuals are themselves unaffected carriers, and that they have a 25% risk of having an affected child.

Bassen-Kornzweig syndrome is a rare genetic disorder that is characterized by an inability to properly absorb dietary fats, resulting in neurological abnormalities, degeneration of the retina of the eye, a typical red blood cell abnormality.

Due to the inability to digest dietary fats, the diet of persons with Bassen-Kornzweig syndrome should contain no more than five ounces of lean meat, fish, or chicken per day.

Treatment is based solely on monitoring the diet and treating symptoms as well as any biochemical abnormalities that might develop. Currently, there is no cure.