The purpose of this review is to provide an update on current literature as it relates to the impact of unilateral hearing loss for the pediatric population. Current studies further corroborate past research findings which reveal the potential consequences of unilateral hearing loss on spatial hearing, language, and neurocognitive functioning. Deficits among children with unilateral hearing loss may parallel those seen in children with bilateral hearing loss, further challenging historic views that hearing in one ear in childhood is sufficient for normal development. The potential deficiencies seen in children with unilateral hearing loss can be subtler than those seen with bilateral hearing loss, but may nonetheless also impact school performance, patient fatigue, parental stress, and quality of life. Early interventions within a sensitive period of development of the central auditory system may circumvent potential barriers in cognitive, academic, and psychosocial development. This review synthesizes the latest research on the impact of unilateral hearing loss in childhood and the role for possible interventions. The summarized information may serve to support the development of new guidelines for the evaluation and treatment of children with unilateral hearing loss.