An indistinguishable clinical presentation of acute neurovisceral attacks is also caused by heterozygous mutations in the genes for either coproporphyrinogen oxidase (CPOX) or protoporphyrinogen oxidase (PPOX), leading to hereditary coproporphyria (HCP, # 121300) and variegata porphyria (VP, # 176200), respectively [1,2,3] (Figure 1). The gene discussed is PPOX; the disease is hereditary coproporphyria.