The signs and symptoms of intestinal obstruction in the newborn often present a difficult diagnostic problem. Neonatal small left colon syndrome is a cause of functional colon obstruction in newborn. It is manifest in the first 24-48 hours of life, particularly in infants of diabetic mothers and, if detected early, it can be fully cured by radiographic contrast enemas. The etiology of the disease is unclear. In this report we describe a case with neonatal small left colon syndrome, treated by daily enemas, and we also reviewed the etiologic explanations for the disease in the literature.