Here, we describe that a homozygous hypomorphic OTULIN mutation in a consanguineous family causes a potentially fatal autoinflammatory disorder termed OTULIN-related autoinflammatory syndrome (ORAS), which can be managed by Infliximab (anti-TNF neutralizing antibody). This evidence concerns the gene OTULIN and autoinflammation, panniculitis, and dermatosis syndrome, autosomal recessive.