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The aim of the study was to present a woman affected of a narcolepsy with cataplexy (narcolepsy type 1) comorbid with an asymptomatic Primary Biliary Cholangitis (PBC). The HLA haplotype was DRB1*15:01, DQA1*01:02, DQB1*06:02. The allele DQB1*06:02 has been considered until now protective for PBC and dual pathology has not been published. We think the important clinical message of the Case would be of continuing to monitor adults with narcolepsy type 1 for late complications that may be associated with other autoimmune conditions. Clinicians should be aware of the relationship between Narcolepsy and PBC. This highlights the need for screening and management in these individuals. • We have published for the first time that autoimmune diseases (AD) are frequently associated in NT1 (JOSR, 2014). We demonstrated a higher frequency of AD in our series of 158 with NT1 patients compared with a sample of the Spanish general population (2016). Finally, in a longitudinal study over 6 years (2022) in our series we found a significant increase of AD. • This findings, together with the hypothesis "epigenetic silencing may represent a key causative factor in several autoimmune diseases" (Seifinejad A et al., 2023), support the idea that narcolepsy is an AD. • The associations NT1 and Primary Biliary Cholangitis observed in this Case is unique and strengthens the hypothesis that similar immune-mediated mechanisms may be involved in both diseases. [ABSTRACT FROM AUTHOR]