Ectomesenchymal chondromyxoid tumor (ECMT) is a rare benign intraoral tumor. Clinically, it presents as a slow growing, painless, firm, submucosal sweling exclusively occurring on the anterior dorsum of the tongue. Till date only 40 cases have been reported in literature. Histopathologically the tumor is characterized by a well circumscribed, lobular proliferation of round, polygonal, ovoid or fusiform cells in a net-like pattern in a myxoid to chondromyxoid background. Here, we present a rare case of ECMT occurring in a 7-year-old boy and throw some light on this distinct entity.