Abstract Lipoblastoma and lipoblastomatosis are rare benign tumours of embryonal fat that exhibit a tendency to invade locally but not to metastasise. This condition most often presents before the age of 3 years, affects males more than females, and is typified by a slowly-growing, usually subcutaneous mass arising in a limb. Cases involving the head and neck are extremely rare, with only nine cases in the neck having been described to date. This tenth case arose in a 13-month-old male presenting with a rapidly enlarging lump in the right side of the neck, clinically mimicking a cystic hygroma. Diagnosis is by histology, and treatment involves complete surgical resection. We report this tenth known case of lipoblastoma in the neck and review the literature.