Clinics and diagnosis of a rare form of genetically grounded heterotopic ossification, i.e. progressive ossificating fibrodysplasia are presented on the basis of literature review and the authors experience. The authors described one patient, age 4,5 years, with heterotopic issificates on occipitocervical, subscapular and axillary zones. The patient had anomaly of great toes, flattened cervical vertebrae and deformity of the the fingers. The diagnosis of this syndrome is based on following clinical and roentgenologic criteria: presence of congenital deformity, shortening of toes and heterotopic ossification of soft tissues. Effective method of treatment does not exist.