Hereditory Sensory Automnomic Neuropathy (HSAN) is a rare disorder. Five types of HSAN have been described by Dyck et al. Congenital insensitivity to pain with anhidrosis is classified HSAN Type IV. The clinical features include inability to sweat, causing defective thermoregulation, and congenital analgesia, which leads to orthopedic complications such as osteomyelitis, fractures, self mutilation and neuropathic arthropathies. Abnormal findings on nerve biopsy, virtual absence of unmyelinated nerve fibers, have been reported. We report a 5-year-old boy who presented with congenital insensitivity to pain, anhidrosis with fracture of lateral condyle of humerus and its complication.