We report the results of the first systematic review of the worldwide literature on eruptive vellus hair cysts (EVHC). It is likely that EVHC are less rare than it may appear from the scarcity of related publications in the literature. EVHC may be present at birth and may appear at any age, although they show a clear trend towards occurring during the first 3 decades of life. A strong clue to the heavy influence of genes on the occurrence of EVHC is provided by the numerous reports of families in whom two or more members were affected. EVHC lesions present clinically in a rather monomorphous fashion, i.e. round, dome-shaped, skin-colored, asymptomatic, soft-tender papules with a smooth surface and grouped or disseminated in a symmetric pattern. EVHC may affect any cutaneous area, even if the upper part of the body and some distribution patterns are particularly frequent and recognizable, i.e. cephalic, upper trunk around the midline, upper limb including axillae, and proximal lower limb. Such a distribution is likely not random and seems to grossly overlap with that of pilosebaceous and apocrine units. Like clinical morphology, the histologic features of EVHC papules are rather monomorphous, indeed, the diagnostic hallmark being the presence of vellus hair shafts within the cystic space. Peculiar subgroups (familial, late-onset, unilesional, and associated with steatocystoma multiplex) are also identified and discussed. In conclusion, EVHC are basically a cosmetic concern to patients but represent a chronic and difficult-to-treat condition. On the basis of our review, future studies are warranted, mainly concerning (i) further nosographic framing involving genetic and tissue analysis, (ii) implementation of non-invasive diagnostic procedures, and (iii) therapeutic trials of interventions shown to achieve some effectiveness.