Darier’s disease (dyskeratosis follicularis) is a rare disease with an autosomal dominant type of inheritance, caused by heterozygous mutation of the ATP2A2 gene. It is usually developed during the first or second decade of life in persons with positive family history. The disease is clinically characterized by follicular keratotic papules, covered with yellow scales or crusts, which fuse into plaques, localizing in seborrheic areas: on the scalp, face, torso, neck’s lateral surfaces. Less commonly, papular eruptions are located on the dorsum of the hands and feet, in the axillary and inguinal folds, on the oral mucous membrane. Hyperkeratotic lesions on the skin of palms and soles, changes in nail plates can be observed. In most cases, there is either isolated lesion of seborrheic areas, or a combination of eruptions in seborrheic areas with manifestations of other localization. We present a follow-up of a patient with a negative family history who noted the appearance of eruptions in the area of scrotum and inguinal folds, accompanied by moderate itching at the age of 35. The process was localized and considered by the dermatologist to be microbial eczema for three years. The lack of positive response to the therapy was the reason to contact the clinic. The clinical pattern (follicular papules fusing into plaques covered with crusts) and dermoscopic examination, in which polygonal brown structures with a white rim on the periphery were observed, surrounded by linear and dotted vessels, helped to suggest keratosis follicularis. The final diagnosis was made based on the detection of suprabasal acantholysis and rounded cells in the spinous layer of acanthotic epidermis during morphological examination.