Thyroglossal duct cysts are the most frequently encountered congenital abnormality of the neck, and are described extensively in the literature. The high incidence of this abnormality and its presence in all age groups requires clinicians to be aware of the clinical features, etiology, and current treatment options. Because it is readily accessible, relatively inexpensive, and non-invasive, sonography is arguably the ideal initial investigation. Computerized tomography and fine needle aspiration biopsy are often utilized as supplementary techniques for confirmation of the diagnosis. The treatment of choice for thyroglossal duct cysts continues to be the classic Sistrunk procedure, developed in 1920, although several modifications have since been employed. In this review, we aim to explore the embryological development, clinical presentation and diagnostic features of thyroglossal duct cysts. In addition, a useful adaptation to the Sistrunk procedure and the recent application of OK-432 therapy as a promising alternative for treatment of thyroglossal duct cysts will also be discussed.