The decrease in the prevalence of Helicobacter pylori (Hp) infection in children in the world gave rise to a new pathological finding termed as Hp-negative gastritis. Unfortunately, the term “Hp-negative gastritis” has not been identified as a pathological process and has the status of a “second cousin”; in most publications it was never mentioned as a subject to be dealt with, but was “left over” data that was never the topic of the manuscripts’ discussions. Only recently has the topic captured the attention of the pathologists who described this phenomenon in adults, yet the pathological and/or clinical spectrum or significance of this phenomenon has not been adequately investigated. In the current manuscript we describe Hp-negative gastritis in children, summarize its clinical prevalence and touch upon the possible etiology, pathology, and/or therapeutic implication. Overall, this review has concluded that Hp-negative gastritis is a pathological phenomenon in children that needs further investigation, and to date, as the title suggests, is a new clinical enigma that needs to be considered.