Juvenile granulosa cell tumour (JGCT) is a rare benign stromal cell tumour of the testis accounting for approximately 1% of all paediatric testicular tumours. Presenting primarily as a painless testicular mass, the tumour may be associated with undescended testis, hydrocele or testicular torsion. Abnormal karyotype has also been described. We describe an unusual case of a neonatal juvenile granulosa cell tumour presenting as an acutely swollen, tender testis, originally diagnosed as an acute hydrocele. We describe the management and review the literature pertaining to this rare differential diagnosis of a neonatal acute scrotum.