Nicolau syndrome (NS) is a rare injection site reaction, following intramuscular injection of drugs characterized by severe pain, skin discoloration and varying level of tissue necrosis. The case outcomes vary from severe pain, atrophic ulcers to sepsis and limb amputation. We describe a case of the five-year-old girl with diagnosis of NS after intramuscular benzathine penicillin injection. The case was complicated with above the knee amputation of lower limb. This case report intends to remind clinicians that such rare cases should always be thought of in all patients receiving whatsoever drug via intramuscular injections.