Congenital self-healing reticulohistiocytosis (CSHRH) is a rare type of Langerhans cell histiocytosis with potential for relapse and systemic involvement. Whereas CSHRH was traditionally considered a benign disease, there is an approximately 3 percent risk of mortality and a 10 percent chance of relapse. This article, using an extensive review of cases since Hashimoto and Pritzker first described the condition in 1973, highlights the various presentations of CSHRH and reveals high rates of relapse and systemic involvement in cases that specifically address features of CSHRH occurring within the first year of life. The findings from this review will highlight the importance of considering LCH in the differential diagnosis when evaluating a neonate with congenital skin eruptions. Timely diagnosis of CSHRH and treatment of systemic involvement may decrease the likelihood of adverse outcomes. These patients may require closer follow-up and monitoring than previously recommended, especially in the first year of life when relapses and systemic involvement occur most frequently.