<p>Corneal endothelial cell dysfunction is a common cause of corneal decompensation in elderly. We describe a non-guttata corneal endothelial dystrophy with correlation of the clinical features to the histopathological and ultrastructural characteristics in a cohort of Saudi patients. A retrospective study of all consecutive cases of primary corneal decompensation in phakic eyes due to endothelial attenuation, in the absence of guttata is conducted. Patients were treated by either penetrating keratoplasty (PKP) or Descemet’s Stripping Automated Endothelial Keratoplasty (DSAEK) as a primary procedure between 2002 and 2016. Clinical and demographic data were obtained through chart review and the histopathological data were collected by reviewing Descemet’s membrane (DM) in the corneal tissue samples of the affected eyes. We included 17 eyes from 17 patients (10 females and 7 males), with a mean age of 67.17 ± 8.98 years. All patients were phakic, and decreased vision was the patients’ main complaint at presentation. The pre-operative endothelial cell count was obtained in 3 eyes with a mean of 531.7 ± 309.5/mm². Histopathology of the 17 corneal specimens showed thick multi-laminated DM and attenuated endothelium. The success rate of the primary procedure was 70%. The cornea of the other eye remained clear in 12/17 patients. This is a type of corneal endothelial cell dysfunction with a late onset of presentation, spontaneous corneal decompensation in phakic eyes or a rapid onset of decompensation following uncomplicated surgery. It seems to be asymmetrically bilateral among our Saudi patients. Fellow eyes are at the same risk of decompensation. DM lacks the presence of guttata clinically and histopathologically.</p>