The past decade and a half has seen marked changes in the epidemiology of sudden infant death syndrome (SIDS). The avoidance of certain risk factors such as sleeping prone and cigarette smoke exposure has resulted in the death rate falling dramatically. Careful evaluation of environmental factors and endogenous characteristics has led to a greater understanding of the complexities of the syndrome. The development and implementation of death scene and autopsy protocols has led to standardization in approaches to unexpected infant deaths with increasing diagnoses of accidental asphyxia. Despite these advances, there is still confusion surrounding the diagnosis, with deaths being attributed to SIDS in many communities and countries where death scene investigations and autopsies have not been conducted. The following review provides a brief overview of the historical background, epidemiology, pathology, and pathogenesis of SIDS. Contentious issues concerning the diagnosis and current problems are discussed. Despite calls to abandon the designation, SIDS remains a viable term for infants who die in their sleep with no evidence of accident, inflicted injury, or organic disease after a full investigation has been conducted according to standard guidelines.