This article describes a rare dermatosis — Kyrle disease (hyperkeratosis follicular and parafollicular, penetrating the skin) in a 52-year-old patient without concomitant conditions of internal organs. Patient was diagnosed with follicular and parafollicular hyperkeratosis penetrating into the skin 13 years after the onset of the disease. The patient received a number a skin biopsies with histological examination. Characteristic clinical and patomorphological changes are presented. Differential diagnosis with the following dermatoses is discussed in detail: hyperkeratosis lenticularis perstans (Flegel’s disease; H. Flegel, 1958), perforating intrapapillary elastosis (J. Miescher, 1955) or elastosis perforans serpiginosa (W. Lutz, 1953), acne vulgaris and acne inversa, follicular dyskeratosis (morbus Darier), pityriasis rubra pilaris (Devergie’s disease), lichen planus follicularis, multiple keratoacanthoma and disseminated superficial actinic porokeratosis. Attention is drawn to the difficulties of diagnosis. We were unable to confirm the follicular localization of hyperkeratotic papules in this patient. A good therapeutic effect was obtained during the treatment with isotretinoin, which gave way to relapse after a significant decrease in the dosage of the drug.