We report two new cases of HIV-1 seropositive subjects who developed porphyria cutanea tarda (PCT). Laboratory examinations revealed a rise of uroporphyrin in the 24-hour urine and a fall of CD4 lymphocytes below 200/mm3 reflecting severe immuno-depression. Treatment with iterative phlebotomy resulted in regression of PCT. In both patients HIV seropositivity was discovered before PCT was diagnosed. Up to now, none of the hypotheses put forward concerning the exact role played by HIV in the genesis of PCT has been confirmed.