A 20-year-old male with bilateral osteochondritis dissecans (OD) of the distal lateral femoral condyle is presented. OD can occur in many joints; however, the medial femoral condyle of the knee is the most common. Bilateral OD of the distal lateral femoral condyle is extremely rare. In our case, the lesion on the right side was traumatic and the lesion on the left side was self-induced. We performed open surgery for both knees. The fragments were stabilized with multiple Herbert screws. This case supports the theory that a defect in the ossification center of the distal lateral femoral condyle plays a role in the etiology of osteochondritis dissecans.