The term “primary intraosseous squamous cell carcinoma” was introduced in 2005 by the World Health Organization with three subcategories. Squamous cell carcinoma arising from the lining of an odontogenic cyst is one important rare subcategory of such lesions with an incidence of 0.01 to 0.02%. Furthermore, the appearance of such malignancy in an odontogenic tumor such as keratocystic odontogenic tumor is considered extremely rare. In this case report we report a case of a 50-year-old Sri Lankan woman who complained of pain and increase in the size of a swelling at the anterior mandible, which had been present for over 1 year. The increase was significant for 1 month with accompanying numbness of the left half of her lip. Cone beam computed tomography results revealed an irregular radiolucent lesion involving most of her mandible and, except in the anterior part, very little buccolingual expansion was seen that suggested a keratocystic odontogenic tumor. An excision biopsy of the cyst lining confirmed a squamous cell carcinoma arising from a preexisting keratocystic odontogenic tumor. Even though primary intraosseous squamous cell carcinoma arising from a keratocystic odontogenic tumor is considered to be very rare, the present case is comparable to most of the aspects cited in the literature. The current case emphasizes the importance of careful investigation of swellings present in the mandible. Clinicians as well as patients should be aware and detect these changes to avoid being clinically negligent.

Congenital anomalies of tongue are rare disorders usually associated with syndromes. In present article we describe a case of a non-syndromic adult patient with bifid tongue with salivary hamartoma on the dorsum of the tongue. We described the clinical features, problems faced by the patient and management. As etiology is uncertain we should have proper understanding of embryology.