Ectomesenchymal chondromyxoid tumor is a rare benign tumor. It occurs uniquely on the oral cavity particularly the tongue. It was first described in 1995, and only 36 cases were reported in the literature. We report a case of ectomesenchymal chondromyxoid tumor in a 67-years-old male, who presented with an asymptomatic, firm, round shaped mass on the dorsum of the tongue. The lesion was completely excised and confirmed as ectomesenchymal chondromyxoid tumor on histopathological examination.