The absence of a cerebellum is extremely rare condition and the clinical presentation is variable. We aimed to present a case with isolated cerebellar agenesis in a pregnant woman at 23 weeks of gestation. Twenty seven years old primigravid patient was referred to our obstetrics outpatient clinic with a suspicion of cerebellar in her fetus. Cerebellum agenesis has been identified by ultrasound evaluation and magnetic resonance imaging. The family was informed about cerebellar agenesis. After counseling and taking written informed consent, pregnancy was terminated. In conclusion, a termination choice may be presented to a family with the early diagnosis of isolated cerebellar agenesis.