Epithelial cysts are benign epithelium-lined lesions that contain fluid or semisolid material. Most epithelial cysts in the oral cavity occur in the anterior part of the mouth floor. Cysts arising on the uvula in a cleft palate patient are rare. Intraoral examination in a 14-month-old boy with a complete cleft lip and palate revealed a cystic lesion on the right uvula. The lesion was excised and push-back palatorrhaphy with Z-plasty on the uvula was performed. Histopathological examination diagnosed an epithelial cyst. We report a case of an epithelial cyst of uvula in a patient with a unilateral complete cleft lip and palate.