The exact cause of Kohler disease is unknown.
Some orthopedic specialists think that Kohler disease may be related to an injury in the area around the navicular bone in the foot and may be the result of delayed bone formation (ossification). Bone ossification usually begins at age 18-24 months in girls and at age 24 to 30 months in boys. Structural weakness might result from an increase in the ratio of cartilage to bone. Since the navicular bone is part of the mechanism by which the foot moves (articulation), it is subject to weight-bearing pressures and stresses from twisting and turning.
Under normal circumstances, the navicular bone is served by a blood vessel from which smaller arteries supply blood to the regions of bone growth. At around the ages of 4-6, the blood supply to these regions of bone growth increases as other blood vessels reach them. If ossification is delayed and the child gains weight, the effect is to compress the blood vessels, thus causing tissue destruction (ischemia).
It has been suggested that genetic factors may play a role in the development of Kohler disease, but a specific gene has not been found to be associated with this disease.