We report a case of follicular and parafollicular hyperkeratosis penetrating the skin (Kyrle disease) in a 44 year old male. We present a literature review, providing rationale for genetically determined processes of desynchronization of keratinocyte differentiation with respect to their proliferation. We describe the clinical and histopathological features of the disease, the methods of differential diagnosis, and treatment strategies, including possible administration of systemic retinoids.