Bilateral stromal corneal opacity is a differential diagnostic challenge for ophthalmologists. In this article 2 female patients aged 30 and 36 years old, respectively, with different degrees of expression of stromal diffuse corneal opacity will be presented. Patient 1 was of short stature (114 cm) and patient 2 was 172 cm in size. Both patients exhibited altered joint structures of the hands and feet and diffuse stromal corneal opacity. Furthermore, patient 1 had both mitral and aortal insufficiencies and patient 2 an aorta insufficiency. The stromal diffuse corneal opacity was indicative of Schleie syndrome. For patients with reduced vision a lamellary keratoplasty is to be recommended.