A newborn female was referred with the following clinical symptoms: median tongue cleft, palate cleft, ankyloglosson, sublingual intraoral hamartoma and palatal intraoral hamartoma. Magnetic resonance imaging showed a subcutaneous cyst overlying the manubrium sterni. Genetic investigation (chromosome analysis) showed no aberrations and/or variations. The quick growth of the intraoral hamartoma required an excision at the age of 3 months. Under general anaesthesia the intraoral hamartoma was excised and the median tongue cleft was closed. Median tongue clefts are reported to be only associated with orofacial digital syndromes type I, II, IV and VI. If the clinical appearance is described without any association to an orofacial syndrome, the Tessier 30 cleft definition could be used as the best explanation of the symptoms. This is the first description of a combination of tongue cleft, palate cleft, intraoral hamartoma and subcutaneous cyst overlying the manubrium sterni. The clinical symptoms of this patient can be described best as a mild form of an orofacial digital syndrome type II or variation of a Tessier cleft No. 30.