Sialolithiasis is the most common salivary gland disease accounting for more than 50% of the cases. Majority of salivary calculi occur in the submandibular gland and its duct. It has male predilection and is often seen in adults. Majority of the calculi are less than 10 mm in size. Calculi > 15 mm in size are considered giant. Giant sialoliths within the parenchyma of the salivary glands are frequently reported in the literature, but they are uncommon in the salivary ducts. The purpose of this article is to report a case of giant sialolith in the Wharton's duct of a 65-year-old male, which had caused sialo-oral fistula. Literature in English language on giant sialolith in Wharton's duct is reviewed since 1990. Also etiology, pathogenesis, clinical features, diagnosis and management aspects are discussed.