An overview of pathogenesis, clinical presentation and diagnosis of pustular psoriasis is presented in this paper. A clinical case of idiopathic von Zumbusch pustular psoriasis in a 9-year-old child is reported. The disease was characterized by acute onset, severe undulating course, and resistance to the systemic therapy. The sudden onset of illness was accompanied by fever, erythroderma and polylymphoadenopathy. Treatment included various approaches and methods, their combinations, substitution and alternation. The prescription of systemic corticosteroids, cytotoxic drugs (methotrexate) and aromatic retinoids (neotigason) was of paramount importance. Drug doses were appropriate for patient's age. The undertaken complex therapy resulted in a positive clinical effect within 3 months. Clinical remission of the skin process was observed during the next 4 months after the end of treatment.