ABSTRACTCampomelic syndrome is a heterogeneous group of disorders characterized by short‐limbed dwarfism with congenital bowing or bending of the long bones particularly in the lower limbs and is frequently associated with a variety of non‐skeletal manifestations. Two distinct types are (1) long‐limbed campomelic dysplasia with bent bones of normal length and width: (2) short‐limbed campomelic dysplasia in which the bent bones are short and wide. We reported an autopsy case of this syndrome.This male baby with gestational age of 32 weeks was born to a mother with placenta previa, showing abdominal distention and short‐limbed appearance. Postmortem examination revealed bilaterally enlarged Potter type 1 polycystic kidneys, a pancreatic cyst, and two spleens in the abdominal cavity. Both lungs were hypoplastic and the heart exhibited multiple congenital anomalies such as double outlet right ventricle, pulmonary artery hy‐poplasia, mitral atresia, absence of ductus arteriosus, and inferior vena cava draining into the left atrium. Characteristically both femur bones were curved at proximal two‐thirds with slightly shortened lengths. Both humeri also showed bending of distal one‐third. Other chondro‐osseous abnormalities such as small epiglottis, narrow thorax, dolichoce‐phaly, and bilateral equinovarus deformity were noted. These findings were compatible with campomelic dysplasia of long‐limbed type accompanying unusual sets of non‐skeletal manifestations of Potter's syndrome and cardiosplenic syndrome. Key words: campomelic syndrome, Potter's syndrome