We report on a 10-year-old Japanese girl with Robinow syndrome accompanied by partial growth hormone (GH) insufficiency. We started GH replacement therapy at the chronological age of 6.5 years. In this case, improvement in the growth velocity was remarkable, but bone maturation accelerated even more. In order to avoid further acceleration of bone maturation, we started to treat our patient at the chronological age of 9.8 years with GH combined with gonadal suppression therapy using a luteinizing hormone releasing hormone analogue. However, no improvement in height SDS for bone age was attained. Our observations suggest that the indication of GH therapy for patients with Robinow syndrome needs careful consideration.