Combustion pathology remains one of the most topical and social important problems of practical public health. A special category of burned patients are children with numerous comorbidities. Purpose - to present the clinical case of successful management of severe burn victim – the child with numerous birth defects and comorbidities. Materials and methods. A 7-year-old male patient V. The diagnosis «Third-degree flame burn, 45% of the total body surface area in the regions of upper extremities, buttocks, lower extremities. Mummification of the feet and legs - to the middle third. Inhalation injury. Burn disease. Burn shock, grade IV. Cerebral palsy. Unbalanced chromosome pathology. Statokinetic development delay. Psychomotor development retardation. Hyperhomocysteinemia. Muscle hypotension syndrome. Acute gastritis. Congenital heart disease. Arachnoid cyst. Partial optic nerve atrophy. Concomitant convergent strabismus. Right-sided abdominal cryptorchidism. Aphthous dermatitis. Polyvalent food allergy». The patient underwent early necrectomy-amputation, three necrectomies followed by wound closure with xenoderm grafts, and four skin autografting procedures. Results. Extremely severe thermal trauma in patients with serious congenital pathology was previously thought by experts to be the injury incompatible with life. In the clinical case presented, the patient’s life was saved due to early surgical treatment, which included guillotine amputation-necrectomy of lower extremities aimed at radical debridement to prevent the development of severe burn disease, along with appropriate infusion and pharmacological therapy. Subsequent surgical procedures stabilized the patient’s condition, decreased the severity of injury, arrested the progression of burn disease, thus leading to his recovery. By the time of discharge from the hospital (on day 91 post injury), the wounds had completely healed, laboratory parameters were within normal limits. Conclusions. The prognoses like “the injury is not compatible with life, and treatment is symptomatic" should not be pronounced by clinicians in general and by burn experts in particular. To save the patient’s life by all possible means should be the primary goal for all medical professionals. The research was carried out in accordance with the principles of the Helsinki Declaration. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors. Keywords: burns, children, congenital malformations, concomitant pathology, treatment.