Weill Marchesani syndrome (WMS) is a rare condition characterized by short stature, brachydactyly, joint stiffness, and characteristic eye abnormalities including microspherophakia, ectopia of the lens, severe myopia, and glaucoma. We report a typical case of WMS in an 18-year old female Ethiopian patient who presented with progressive visual loss from chronic angle closure glaucoma secondary to lens subluxation. Patients with WMS require early diagnosis in order to treat serious ocular and life-threatening systemic complications. To the best of our knowledge, only one case of the syndrome was previously reported in Africa.