Porphyria cutanea tarda (PCT), the most common of the porphyrias, is a mainly acquired disease of the liver, which manifests with bullous skin lesions. However, up to 20% of patients with PCT, usually those with chronic untreated disease, are reported to develop some sclerodermatous changes that may affect both light-exposed and nonexposed areas and that can be histologically indistinguishable from true scleroderma. A small number of patients with PCT has severe or generalized scleroderma, which is not necessarily due to coexistent systemic sclerosis. There are few reports in the literature that detail whether the severe sclerodermatous changes respond to control of the porphyria. We report a case of familial PCT with associated severe sclerodermatous changes causing scarring alopecia, cicatricial ectropion and skin thickening over the upper trunk. The scleroderma improved slightly over a 4-year follow-up period after treatment to normalize porphyrin excretion and prevent relapse.