Background: Association of congenital myopathy with COVID-19 – associated peripheral neuropathy is not reported. We aim to present such a case. Material and methods: Clinical case of a 14-year-old male with history of myopathy from the age of 5 who presented with features of COVID19-related polyneuropathy and multiple organ dysfunction syndrome. Results: The patient was admitted with generalized muscle weakness, motor difficulties, unsteady gait, chest pain, respiratory failure. Physically – hyposthenic body type, muscle atrophy, cyanosis, shortness of breath, tachycardia, hepatomegaly. Neurologically – muscle pain on palpation, hypotonia, especially in lower limbs, distal hypoesthesia, loss of deep tendon reflexes and myopathic gait. IgG and IgM SARS-Cov-2 were elevated and the patient presented a history of fever one month before admission. The albumin and total protein were low, but serum creatine kinase, creatine kinase-MB, LDH, liver enzymes, D-dimers were elevated, as well as cerebrospinal fluid protein level. The chest CT showed fibro atelectasis of S3 and S10 segments of the left lung, pleural adhesions. Electromyography studies showed a myopathic pattern. The patient received five plasma exchange treatments and was weaned from mechanical ventilation. The treatment also included antibiotics, infusion therapy, dexamethasone, which resulted in a partial response. Conclusions: The presented case of the association of congenital myopathy and Covid-19 associated peripheral neuropathy had a partial response to treatment. Such cases should be tailored by a multidisciplinary management team