A 2-year-old presents with an enlarging parotid mass found to be a sialoblastoma on incisional biopsy. She underwent total parotidectomy and infratemporal fossa clearance with subsequent adjuvant chemotherapy. At 5-year follow up, she is disease-free having undergone costochondral TMJ reconstruction and facial nerve reanimation. Sialoblastoma is a rare salivary gland tumour presenting in early childhood. This study aims to review the available literature on sialoblastomas to inform contemporary practice. A systematic review was performed in accordance with Preferred Reporting Items for Reviews and Meta-Analysis (PRISMA) guidelines. Articles describing cases of sialoblastoma were identified and data regarding presentation, investigation, management and outcomes were extracted. Sixty-five articles describing 91 cases met inclusion criteria. Age at initial presentation ranged from birth to 83 years with 70.1% of patients aged below one year with no sex predilection (1:1.1, F:M). Tumours were most commonly in the parotid region followed by submandibular region. Ultrasound or MRI features look benign due to a lack of infiltrative borders. Surgery was usual first line treatment. Eleven patients suffered metastasis, and six patients had synchronous hepatoblastoma. Patients were followed for an average of 4.2 years with seven reported deaths (7.6%) and 22 (24.2%) experiencing recurrence on average 19.1 months after initial treatment. Sialoblastoma should be considered as a differential in an infant or toddler presenting with a salivary gland mass. Soft tissue imaging may lack aggressive features and subsequent tissue diagnosis via an incisional biopsy would be essential. Complete resection with clear margins should be the goal of therapy with adjuvant chemotherapy if indicated. Additionally, patients should be screened for synchronous hepatoblastoma due to high rate of synchronicity. Close oncosurveillance is recommended due to high recurrence within two years.