Introduction: EEC syndrome is a very rare entity and combines multiple deformities of the extremities with cleft formations and anomalies in the mouth, jaw, and face. The syndrome was first described by Eckoldt in 1804. The cause is based on a heterozygous mutation of tumour protein 63 (TP63). Furthermore, TP63-related diseases can be divided into six categories: EEC, AEC, ADULT, LMS, RHS, and SHFM4 syndrome. Material & Method: These are two case documentations in which EEC syndrome was confirmed after human genetic clarification. From an oral surgery point of view, there was a pronounced growth deficit of the midface, the jaw bases, and the alveolar process due to the double-sided cleft palate with missing intermediate jaw and hypodontia. The preprosthetic surgical treatments included dysgnathic, augmentative, mucogingival, and implant surgery. In particular, the hypoplastic V-shaped upper jaw posed a great challenge due to the absence of the intermaxillary jaw. Summary: The regaining of oral quality of life through functional rehabilitation of the stomatognathic system requires complex interdisciplinary cooperation between the specialist disciplines of maxillofacial surgery, orthodontics, and prosthetics. Discussion: People affected by the complex clinical picture of EEC syndrome can benefit from the treatment in terms of mastication, phonetics, and aesthetics. Keywords: ECC Syndrome, rare disease, hypodontics, dental implants