Circumscribed juvenile pityriasis rubra pilaris (PRP) is a form of PRP that manifests with well-defined erythematous scaly plaques with follicular keratosis mainly over knees and elbows. There are several reports of the association of PRP with other conditions. We report a boy with scattered erythematosquamous skin lesions and follicular hyperkeratotic papules since the age of six years. Skin biopsy was compatible with PRP. He also had hypoparathyroidism and brachyonychia. To our knowledge, this association has not been reported so far.