A macular hole is a defect of the foveal retina involving its full thickness from the internal limiting membrane (ILM) to the outer segment of the photoreceptor layer. The condition was first described by Knapp1 in 1869 in a patient who sustained blunt trauma to the eye. The overall prevalence macular hole is approximately 3.3 cases in 1,000, in persons older than 55 years. Peak incidence of idiopathic macular hole is found in the seventh decade of life, and women typically are affected more than men. Reasons for sex difference are not known. Cardiovascular disease, hypertension, and a history of hysterectomy have been reported as risk factors. This chapter covers the pathogenesis of macular holes, staging of macular holes, etiology of macular holes, and investigations. The natural history of a macular hole varies because of its current clinical stage. It has been reported that around 50% of stage 0 and stage 1 macular hole may resolve both in the anatomic changes and in the symptoms. Stage 2 holes progress in most cases to stage 3 or stage 4, resulting in worsening vision.