Henna is a natural product commonly used for cosmetics, healing, and social occasions in the Middle East and South Asian countries. It usually carries no significant medical complications in a healthy individual. However, henna in a patient with G6PD deficiency can cause serious medical complications, including severe hyperbilirubinemia and hemolytic anemia, due to its oxidative stress on the erythrocyte. This paper reports a previously undiagnosed G6PD deficient neonate who presented with severe hyperbilirubinemia without the classical laboratory findings of hemolytic anemia. In addition, we reviewed the literature and summarized the clinical and laboratory findings of 31 G6PD-deficient pediatric patients with henna-induced hemolytic anemia (HIHA). The reported adverse effects of HIHA included death (N: 2), kernicterus (N: 3), life-threatening hemolytic anemia that required blood transfusion (N: 9), and severe hyperbilirubinemia requiring exchange transfusion (N: 7). Although HIHA in G6PD deficiency is a well-known fact in the literature, we believe it is still under-reported. Given the high prevalence of G6PD deficiency and the widespread practice of henna application, we recommend avoiding it, especially in infancy, until the G6PD status is known. Society awareness should be raised about it.