ROSAH syndrome (retinal dystrophy, optic nerve edema, splenomegaly, anhidrosis, and headache), a monogenic autoinflammatory disease caused by gain-of-function mutations in ALPK1, features both classic NF-κB-associated phenotypes such as anhidrotic ectodermal dysplasia and atypical features not typically associated with NF-κB dysfunction (Kozycki et al., 2022). The gene discussed is NFKB1; the disease is inherited retinal dystrophy.