ImportanceErosive pustular dermatosis (EPD) is a rare chronic inflammatory condition of the scalp and legs that is often difficult to manage. Currently, there are no treatment guidelines.ObjectiveTo systematically assess the existing literature on various treatment modalities and their efficacies when used in the management of EPD.Evidence reviewWe searched PubMed, Cochrane Libraries, Scopus, and clicnialtrial.gov databases for articles in the English language with no limited time frame. Emphasis was placed on articles that reported on treatment for EPD.FindingsOf the 168 articles identified by the literature search, 92 met eligibility criteria and were included for qualitative analysis. Efficacious topical treatments included clobetasol, betamethasone, and tacrolimus. Ninety‐three and 88% of cases utilizing clobetasol and betamethasone respectively demonstrated improvement or resolution. All 32 cases utilizing tacrolimus reported improvement. Efficacious systemic treatments included oral steroids such as prednisone, methylprednisolone, and dexamethasone. Topical dapsone, photodynamic therapy, systemic steroids, cyclosporine, and oral zinc derivatives were also described with some success.Conclusions and relevanceAccording to available data, limited solely to case reports and case series, potent topical steroids are an effective treatment option for EPD. Topical tacrolimus may also be considered in cases that require long‐term use or maintenance. Other treatment modalities shown to be successful based on high reported efficacy and low rates of recurrence after treatment include topical dapsone, systemic steroids, zinc derivatives, and cyclosporine. Further studies are needed to compare treatment modalities and to establish treatment protocols.