Onychomadesis is characterized by separation of the nail plate from the matrix with persistent attachment to the nail bed and often, but not always, eventual shedding. Onychomadesis has been associated with infection, autoimmune disease, critical illness and medications. To our knowledge a literature review of all associations with onychomadesis has not been completed previously. Most commonly, onychomadesis has been reported in association with pemphigus vulgaris and hand-foot-mouth disease, and following chemotherapy or antiepileptic medications. This article summarizes these key culprit associations, postulates the pathogenesis of nail matrix arrest and summarizes the clinical outcomes. We conducted a retrospective review of cases of onychomadesis reported from January 1960 to March 2013. Using the PubMed database, the literature was searched using the following terms: 'onychomadesis' and 'proximal nail shedding'. Also, an Ovid search was carried out using the same terms. In total 56 articles have been published, including our previously reported series of idiopathic onychomadesis. Articles pertaining only to Beau's lines and not true onychomadesis were excluded. Onychomadesis has been associated with autoimmune disease, other major medical illness, neonatal illness, medication and infection.