Background: Dermoid cysts are benign congenital lesions occurring in about 80% in the ovaries and testicles and only in about 1.6-7% are they found in the head and neck region, mainly in the midline of the body.Case report: A rare case of congenital dermoid cyst in the central compartment of the neck is presented. It was diagnosed when the patient was 10 years old; later at age 22 the patient was re-diagnosed and qualified for surgical treatment. The paper outlines the diagnostic and therapeutic procedures performed. It demonstrates the value of appropriate tests and exclusion of other possible diseases.Conclusions: Dermoid cysts are a rare disorder affecting mainly the ovaries and testicles which require detailed diagnostics as a part of differential diagnosis. In most cases, they do not cause severe symptoms and often require only simple surgical treatment.