Context: Congenital amyoplasia is characterized by contractures (arthrogryposis) involving multiple large joints. Case report: The patient is a couple’s first daughter and no history of similar cases in the family. She was born at term, by cesarean delivery, weighing 3080 grams and with Apgar scores of 8 and 9. Gestational ultrasound revealed fetal akinesia, oligodramnia, and altered fetal skeletal musculature with shortening of the four limbs. During delivery, she suffered a fracture of the right femur. The patient evolved with a delay in neuropsychomotor development. On physical examination, hypotonia, nevus flammeus on the forehead, contractures involving several joints (including fingers, elbows, hips, knees and feet) were observed as well as pits in elbows and knees. Computed tomography scan of the skull showed cortical hypoplasia. Radiographic evaluation showed levoconvex thoracolumbar scoliosis and congenital changes in vertebral bodies of the thoracic spine, and thinning bone structures of the upper limbs. In addition, there were dysplastic acetabular, signs of constriction or tissue band in distal third of the right and middle thighs of the left, intense muscular hypotrophy, thinning of diaphysis of the femurs and echinovirus feet. Abdominal ultrasound and karyotype were normal. Conclusions: The clinical findings were compatible with those of congenital amyoplasia. Fetal akinesia or hypokinesia is a finding widely reported by mothers of children with this condition, as occurred in our case. Therefore, early interventions are very important, in order to avoid, in particular, greatest atrophy of the affected limbs, in addition to osteopenia.