The association of alpha-1 antitrypsin deficiency (PiZ phenotype) with systemic nodular panniculitis has been well documented. Despite reports of cases of systemic nodular panniculitis associated with other alpha-1 antitrypsin deficiency phenotypes, it is still not known if this association is fortuitous rather than causal. We report a case of systemic nodular panniculitis associated with alpha-1 antitrypsin deficiency (PiSS phenotype), with clinico-pathological features similar to those reported in cases associated with the PiZ phenotype.