Pernio (chilblains) is an inflammatory condition classically characterized by localized erythema and swelling of acral sites upon exposure to cool and damp conditions. It is reported a case of a 59-year-old otherwise healthy woman with acute primary perniosis. She had a 3-day history of lesions on the toes brought on by cold, damp weather. On initial presentation, a biopsy sample was taken of a hallux lesion, and the patient was given a trial course of oral pentoxifylline, topical corticoid and nifedipine therapy. Follow-up at 2 weeks showed complete relief of symptoms and the biopsy results confirmed the diagnosis. Pentoxifylline therapy has been shown to be effective and should be considered the standard of care in the treatment of perniosis along with conservative environmental measures.The etiology and pathogenesis of perniosis are reviewed and discussed, as well as differential diagnoses and treatment options.