Major salivary gland absence is a rare disorder. The cause of congenital absence of the salivary glands has not been determined, but it may be associated with ectodermal defects of the first and second branchial arches. Isolated absence of a unilateral submandibular gland is an unusual entity with less than ten cases reported in the literature. The etiopathogenesis of isolated absence of a major salivary gland without other developmental anomalies is still unclear. The formation of a sialolith within the remaining Wharton?s duct, associated with isolated aplasia (versus atrophy) of a unilateral submandibular gland has been recently reported. We describe in this work two cases of sialolithiasis within the ipsilateral remaining Wharton´s duct in patients with isolated absence of a unilateral submandibular gland. In the cases reported, absence of the submandibular gland may have been the result of the complete acinar atrophy secondary to an early obstruction of Wharton´s duct.