Aim: To report a case of a patient with pityriasis lichenoides et varioliformis acuta, emphasising the challenges in the diagnosis and treatment of this clinical entity. Case report: A 30-year-old patient was referred to the Department of Dermatology and Venereology due to skin changes accompanied by itching persisting for one and a half month. Red papules and papulocrusts were found on the trunk, upper and lower limbs, but in a larger number and more severely affecting the trunk than the limbs. The patient stated the occasional use of febuxostat for the treatment of chronic hyperuricemia that has been diagnosed four years prior. Treatment with desloratadine and a combination of bethametasone and gentamicin was started. The laboratory results showed neutropenia, high levels of low density lipoprotein cholesterol and high levels of urate. The biopsy of the skin lesion has been done. Doxycycline treatment and continuing treatment with bethametasone and gentamicin were recommended to the patient. Pathohistological finding described ulcerated epidermis with oedema, granulation tissue and erythrocyte extravasation in the floor of the ulceration. Lymphocytic and histiocytic infiltrates were found in the base of the ulceration and subepidermally. The epidermis on the edge of the ulceration was irregularly hyperplastic and showed spongiosis. The diagnosis of pityriasis lichenoides et varioliformis acuta has been confirmed. Upon completion of doxycycline treatment skin changes were in regression and urate levels decreased. Conclusions: Not only caution in diagnosis and treatment, but also regular follow-up is necessary in patients diagnosed with pityriasis lichenoides et varioliformis acuta because of a wide range of differential diagnoses and a possibility of misdiagnosis. Further research on the etiopathogenesis of this disorder is needed in order to improve treatment outcomes.