ROSAH syndrome belongs to the spectrum of relopathies (i.e., diseases impacting NF-κB activity) but differs from most of these diseases13 suggesting that the specific symptoms observed may be due to a specific expression pattern of the ALPK1/TIFA pathway or to a specific regulation of this pathway. The gene discussed is NFKB1; the disease is retinal dystrophy, optic nerve edema, splenomegaly, anhidrosis, and migraine headache syndrome.