Hypertrichosis is characterized by increased growth of the hair, which is beyond the normal variation and in areas that are not predominantly androgen-dependent. Gingival overgrowth, a common gingival disease, can be caused by inflammation, drug use, leukemic infiltration, and malignancies. Tooth eruption disorders can manifest in several clinical conditions, where the oral location, the number of affected teeth, and the etiology of the disorders vary considerably.

This clinical case reports delayed permanent teeth eruption with generalized gingival hyperplasia in a child with a genetic disorder and heredited hypertrichosis. A 10-year-old male patient was referred to the pediatric dentist with the main complaints of delayed eruption of permanent teeth and swollen gums. The child was diagnosed with high-grade hydronephrosis in the right kidney before birth. Genetic tests revealed that the child has a pathological male karyotype 45, XY, with a translocation between chromosomes 13 and 14 inherited from the maternal side. On extraoral examination, dysmorphic facies, a brachyplatycephalic head configuration, coarse facial features, a broad nose, and a short neck were evident. The gingiva was pale pink with a fibrous consistency and extended on all tooth surfaces. The gingival tissue did not bleed on probing. Delayed eruption of permanent teeth was observed, with only twelve primary teeth present.

Rare diseases often show unique dental-craniofacial manifestations, and pediatric dentists, oral maxillofacial surgeons, and orthodontists are responsible for identifying and managing each patient's oral symptoms.