Cystic dysplasia of the testis is a rare benign congenital defect in which the testis is a formation of multiple irregularly shapes cystic spaces, first described in 1973 by Leissring and Oppenheimer. We report a case and review the literature to identify clinically useful associations and management options. Literature review. A comprehensive literature search was performed on electronic databases and search platforms. Only English publications were included. Hand search was additionally performed separately by two investigators, with data extracted from relevant publications included patient characteristics, management workup, associated conditions and outcomes. Case report. A male newborn with VACTERL association was noted to have an enlarged right testis and impalpable left testis. Ultrasonography of the testis revealed cystic lesions and tumour markers were normal. Pole to pole biopsy confirmed cystic dysplasia. Mild involution has been noted at 3 months follow-up. Literature review: 40 publications including 55 cases of CDT were identified. Diagnosis was made at median 4.7 years (range 0-12 years) with ultrasound being the main investigation to achieve diagnosis. Strong associations were found between CDT and ipsilateral renal agenesis (50%), MCDK (15%), VACTERL (10%), renal duplication (3%) and Potter’s Syndrome (6%). The most common management by far was orchidectomy. Of those cases which were treated conservatively with observation, almost all were found to have involution on follow up imaging. Clinicians should be aware of CDT as a rare differential for neonatal testicular enlargement. Management should be conservative due to negligible long term malignancy risk and investigations are recommended to check for known associations which have clinical significance.