Neonatal neutropenia is a critical hematologic condition characterized by abnormally low levels of neutrophils, a type of white blood cell crucial for the body's defense against bacterial infections. Occurring in the first 28 days of life, neonates with this condition are particularly vulnerable to serious infections due to their underdeveloped immune systems. The causes of neonatal neutropenia are diverse, encompassing congenital, acquired, and idiopathic factors, with conditions such as maternal autoimmune diseases, genetic abnormalities, or infections playing pivotal roles. The clinical presentation varies widely, ranging from asymptomatic cases to life-threatening sepsis, demanding a vigilant and timely diagnosis. Diagnostic evaluation includes complete blood counts, bone marrow examinations, and genetic testing, all contributing to a comprehensive assessment of the underlying etiology. Management strategies focus on treating the underlying cause when identifiable, employing antimicrobial therapy to combat infections, and, in severe cases, implementing granulocyte colony-stimulating factor (G-CSF) to stimulate neutrophil production. Neonatal neutropenia necessitates a multidisciplinary approach, involving neonatologists, hematologists, and infectious disease specialists, to optimize patient outcomes. Despite advances in medical understanding and therapeutic interventions, neonatal neutropenia remains a complex and challenging condition, demanding ongoing research efforts to enhance diagnostic precision, therapeutic strategies, and long-term outcomes for affected infants.