Gastrointestinal stromal tumor (GIST) is the most common mesenchymal neoplasm of the gastrointestinal tract. Only 1.5% to 2% of all GISTs are observed in children and adolescents. Most of the pediatric cases are between 10 and 18 years of age, with a median age of 13 years. GIST is extremely rare in the newborn period. We could find only 5 reports on the neonatal cases. Herein, we have reported a case with abdominal tumor that was identified by prenatal ultrasonography and magnetic resonance imaging, and diagnosed as GIST on the seventh day of life. We have also reviewed the neonatal GIST cases reported in the English literature.