The aim of this manuscript is to describe a novel retinal finding of Alport syndrome during surgical management of an associated macular hole. A retrospective chart review of a 65-year-old man with a diagnosis of Alport syndrome confirmed by renal biopsy was found to have an associated full-thickness macular hole. Pars-plana vitrectomy surgery with internal limiting membrane (ILM) peeling was attempted, but intraoperatively the ILM was found to be absent at the macula. Alport syndrome may be associated with the absence of the ILM. This can complicate attempts at macular hole repair.