Neurolipomatosis is a rare benign tumor of peripheral nerves. The nerves of the upper extremities are more often involved, in most cases, the median nerves in the carpal canal. Children and young people suffer mainly. The article describes a case of fibrolipomatous hamarthroma of the median nerve in a 24-year-old patient, the ultrasound image is compared with the results of intraoperative observation. Despite the characteristic clinical and pathomorphological picture of this condition, the issues of diagnosis and differential diagnosis remain relevant. A special role is given to ultrasound as a non-invasive, widespread method of lifetime diagnosis of the condition of peripheral nerves. The median nerve, due to its superficial location, is available for visualization, and understanding the ultrasound semiotics of fibrolipomatous hamartoma will allow timely determination of it at the stage of initial clinical manifestations and avoid unnecessary and aggravating neurological deficits of examination methods such as biopsy.