Hyperimmunoglobulin E syndrome (hyper-IgE) is a rare immunodeficiency disease associated with recurrent pyogenic infections, chronic eczematoid dermatitis and osteopenia. We present here a 13-year-old girl with hyperimmunoglobulin E syndrome, who developed osteochondritis dissecans (OCD) of the lateral femoral condyle, which is rare. Osteopenia, which is frequently associated with hyper IgE, may predispose the patient to the development of OCD.