Background: Keratitis-Ichthyosis-Deafness (KID) syndrome is a rare congenital ectodermal disorder characterized by ichthyosiform skin lesions, sensorineural hearing loss, and ocular involvement, with considerable phenotypic variability and occasional familial occurrence.

Case Presentation: A 20-year-old male presented with generalized scaling and hyperpigmented patches since birth, associated with palmoplantar hyperkeratosis and hearing impairment. A similar clinical presentation was observed in his 11-year-old cousin, who had more extensive cutaneous involvement and earlier onset of hearing loss. Cutaneous examination in both patients revealed generalized ichthyosiform scaling with palmoplantar keratoderma, while systemic findings were unremarkable. Skin biopsy demonstrated marked hyperkeratosis and acanthosis consistent with congenital ichthyosis. Based on clinical features, family history, and histopathology, a diagnosis suggestive of KID syndrome was made.

Conclusion: These cases emphasize the phenotypic variability and possible familial nature of KID syndrome. Early recognition is essential for multidisciplinary management, genetic counseling, and long-term follow-up to prevent complications.