Introduction: A patient with TNRT1 defect with COVID-19 infection, was treated with Nirmatrelvir/Ritonavir, a new drug accepted for the treatment of COVID-19, combined with immunoglobulin.
Presentation of the case: A 30-year-old female patient, from Guzman City, Jalisco, diagnosed in 2022 with TRNT1 heterozygous defect, she has total absence of lung parenchyma. In August 2022 she presented fever and increment in oxygen requirement, she had a SARS-COV2 antigen test, resulting positive, she was admitted in bad general state, dyspnea, she received 25 gr of intravenous Immunoglobulin (IGIV). Due to her poor lung reserve for fibrotic damage, she was considered candidate to antiviral treatment and received Nirmatrelvir/Ritonavir 300 mg/100 mg every 12 hours for 5 days, her oxygen requirement decreased. However, she had aggregate infections: pneumonia by Pseudomonas aeruginosa and clostridioides, she received treatment with Ceftazolane/Tazobactam, Vancomycin, and 20 gr of IGIV, with improvement. About a month later she developed ascites and data of portal hypertension requiring variceal ligation on September 2022 and January 2023, she had been studied for Gastroenterology. She had a hepatic biopsy on May 2023, with a partial report of a mild chronic portal inflammation.

CT: Right hemithorax: total absence of pulmonary parenchyma, dextrocardia. Left hemithorax: cystic, varicose and cylindric bronchiectasis, mosaic pattern.
Laboratory: 08/18/2022: Hemoglobin 8.5 g/dl, HCM 23.8 fl, VCM 80 pg/cel, Leucocytes 6600, Neutrophils 6280, Lymphocytes 160, Platelets 76000. 09/12/22 IgG 856
Hepatic Biopsy: mild chronic portal inflammation, without other relevant data, pending stainings.

Discussion: Several studies have suggested that Nirmatrelvir/Ritonavir might be effective in terms of reducing mortality and hospitalization rate. There are few cases reported with its use in our country, and no cases reported its use in patients with inborn immunity error. This patient was treated and successfully overcame covid-19, subsequently she developed signs of hepatopathy, which is still subject of study.