Gorlin–Goltz syndrome (GGS) is a rare multisystem disorder characterized by autosomal dominant inheritance and age-dependent variability in clinical manifestations. Odontogenic keratocysts of the jaws represent a hallmark feature of the syndrome and, in pediatric patients, may precede the development of other systemic symptoms. Currently, there is no consensus on whether the Marfanoid phenotype is a rare manifestation of GGS itself or a phenotypic co-occurrence of two genetically independent dysmorphologies. This study aimed to highlight the diagnostic significance of multiple jaw keratocysts in the early detection of GGS and to demonstrate a rare clinical association of GGS with Marfanoid-type undifferentiated connective tissue dysplasia in a child. We present a clinical observation of an 11-year-old female patient with recurrent multifocal cystic jaw lesions, GGS, and undifferentiated connective tissue dysplasia of the Marfanoid type. In the reported case, the presence of multifocal and recurrent cystic jaw lesions, progressing since the age of 4, led to clinical suspicion and subsequent confirmation of GGS. Genetic counseling further identified familial undifferentiated connective tissue dysplasia with a Marfanoid phenotype in the proband. Multiple odontogenic keratocysts in children serve as a critical early diagnostic marker for GGS, necessitating a multidisciplinary approach to diagnosis and management. The presented case is distinguished by the early onset of jaw cysts and the concurrent presentation of GGS with Marfanoid-type undifferentiated connective tissue dysplasia.