We report the case of a girl with mosaic Turner syndrome who developed abdominal neuroblastoma during growth hormone (GH) therapy for short stature. The patient underwent chemotherapy for the unresectable tumor but died at the age of 10 years due to multiple metastases, five years and 10 months after the diagnosis of neuroblastoma. Although the risk of neuroblastoma does not increase in patients with Turner syndrome, the need for pre-treatment imaging studies for tumor screening remains controversial. However, our experience with this patient highlights the potential value of performing imaging studies both before and during GH treatment.