ABSTRACT: Pityriasis rubra pilaris (PRP) Type IV, the circumscribed juvenile form, is a rare, chronic papulosquamous disorder in children. It can be misdiagnosed as paediatric psoriasis because both share erythematous plaques and hyperkeratotic lesions. PRP Type IV typically presents with well-demarcated, scaly, reddish-orange plaques on the elbows, knees, hands, and feet. Orange-hued palmoplantar keratoderma may also accompany the condition. Diagnosis relies on clinicopathological correlation, with biopsy typically revealing hyperkeratosis and a checkerboard pattern of alternating parakeratosis and orthokeratosis. Management is challenging because no standardised treatment guidelines exist, although combined topical and systemic therapy may be effective. In this report, a 4-year-old girl presented with a 2-year history of persistent pruritic plaques. She was treated with low-dose oral acitretin, topical emollients, corticosteroids, and vitamin D analogues, resulting in marked clinical improvement and good treatment tolerance. This case highlights the importance of accurate diagnosis and demonstrates that early, appropriately tailored therapy can achieve favourable outcomes and improve quality of life in paediatric patients with PRP Type IV.