Objective To explore the application and effect of knowledge learning and skills competition of medical genetics. Methods A total of 182 students were selected from the 2015 clinical medicine major of Jiamusi University. Among them, class 1 and class 2 including 93 students were experimental groups, class 3 and 4 including 89 students were control groups. By the end of medical genetics course, medical genetics knowledge and skills competition was conducted in the experimental group. The student’s grades in the experimental group consisted of 60% of the final period and 40% of the routine scores. The routine grades consisted of usual attendance and classroom participation discussions (20%), experiment (10%), knowledge and skill competition (10%).By the end of the course, medical genetics knowledge and skills competition was not conducted in the control group. Both groups were evaluated by the same test paper. The student's grades in the control group consisted of 60% of the final period and 40% of the routine scores. The peacetime grades consisted of usual attendance and classroom participation discussions (20%), experiment (20%). The assessment form informs the students in the first class and allows the students to take appropriate learning methods. The teaching effect is evaluated by the two sides of the questionnaire and by the examination results. Results The average score of the experimental group was significantly higher than that of the control group (t=4.47, P<0.01), but the percentage of students who fail to pass the test, there was no statistically significant (χ²=1.04, P>0.05). The results of questionnaire survey showed that most of the students in the experimental group were recognized for medical genetics knowledge and skills competition, experimental group is higher than the control group ( all P<0.01) in the active learning skills of this course; experimental group is higher than the control group ( all P<0.01) in the development of comprehensive ability. Conclusions The development of knowledge and skills competition in medical genetics can stimulate students’ love for the course, cultivate their ability self-learning, clinical thinking and clinical practice.