Purpose: Auditory neuropathy spectrum disorder, or ANSD, can be a confusing diagnosis to physicians, clinicians, those diagnosed, and parents of children diagnosed with the condition. The purpose of this review is to provide the reader with an understanding of the disorder, the limitations in current tools to determine site(s) of lesion, and management techniques. Method: This article is a review of what is known about ANSD. It includes descriptions of assessment tools, causes of ANSD, and patient management techniques. Conclusions: This review is a guide to audiologists, speech-language pathologists, and early interventionists who work with individuals diagnosed with ANSD and/or their families. It highlights the need for more precise tools to describe the disorder in order to facilitate decisions about interventions and lead to better predictions of outcome.