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Autosomal dominant (AD) and recessive (AR) TBK1 deficiencies have been reported in human patients with herpes simplex encephalitis and SARS-CoV-2 pneumonia (AD deficiency) or systemic inflammation (AR deficiency). We describe here a Turkish boy, born to consanguineous parents and homozygous for a loss-of-function mutation of TBK1, who had experienced recurrent vesicular skin eruptions presumably triggered by herpesviral infection and five episodes of viral pneumonia since the age of 2 mo. Whole-exome sequencing identified a biallelic mutation of the TBK1 gene (NM_013254.4: c.922C&amp;gt;T, p.Arg308Ter). The induction of IFNB, IFNL1, IFIT1, and IL6 was abolished or severely impaired in SV40-fibroblasts from the patient following stimulation of the TLR3 or RIG-I\/MDA5-RIG-I pathways. The patient underwent stem-cell transplantation, but unfortunately succumbed to suspected post-viral acute disseminated encephalomyelitis at the age of 2.5 years. AD or AR TBK1 deficiency should be considered in patients with severe viral infections.<\/jats:p>","DOI":"10.70962\/jhi.20250058","type":"journal-article","created":{"date-parts":[[2025,11,14]],"date-time":"2025-11-14T07:21:33Z","timestamp":1763104893000},"update-policy":"https:\/\/doi.org\/10.70962\/jhi.crossmarkpolicy","source":"Crossref","is-referenced-by-count":4,"title":["Recurrent severe viral infection in a child with inherited complete TBK1 deficiency"],"prefix":"10.70962","volume":"2","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8571-2581","authenticated-orcid":true,"given":"Sara Sebnem","family":"Kilic","sequence":"first","affiliation":[{"id":[{"id":"https:\/\/ror.org\/03tg3eb07","id-type":"ROR","asserted-by":"publisher"}],"name":"Bursa Uludag University, Medical Faculty 1 Department of Pediatric Immunology-Rheumatology, , 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