Congenital titinopathy (CT) is a prenatal/infant-onset genetic muscle disorder caused by recessive TTN mutations. We have identified eleven severely affected infants from nine families with convincingly pathogenic truncating and/or splice site mutations in trans. Two pregnancies were terminated due to severe limb contractures and hydrops. Nine infants were born at or near term with severe to profound axial and limb hypotonia and weakness. Pregnancy was complicated by hypokinesia in nine, polyhydramnios in four, and intrauterine growth retardation in four. Six were delivered by caesarean section because of breech presentation and/or fetal distress. Eight were born with no respiratory effort or severe respiratory compromise and required immediate resuscitation / mechanical ventilation. Ten had congenital proximal and distal joint contractures involving all four limbs (nine cases) or the lower limbs only (one case). Three had congenital long bone fractures. Three had atrial and/or ventricular septal defects. Two had clinical signs of encephalopathy likely secondary to birth asphyxia. Seven infants died shortly after withdrawal of ventilatory support (1st to 44th day of life). One remains reliant on full time ventilatory support via tracheostomy (age nine months) while another no longer needs respiratory support (age two years). Muscle biopsies from five cases showed similar features to published CT cases (fibre size variation +/- internalized nuclei +/- cores). A 6th showed sarcomeric disorganisation but no additional abnormalities. One of the four cases who underwent autopsy had complete absence of multiple limb and trunk muscles but relative sparing of the diaphragm. In view of the features present in these patients, we recommend that congenital titinopathy be considered in infants with: (1) marked fetal hypokinesia (2) multiple prenatal-onset limb contractures (3) severe to profound congenital weakness (4) congenital long bone fractures and/or (5) complete absence of muscle groups.