Signet ring cell carcinoma (SRCC) is a rare lesion in the gastrointestinal tract. Further, the condition is very uncommon at the ampulla of Vater. A majority of the reported cases are typical, gland-forming adenocarcinomas. In our case, a patient aged 59 years, was diagnosed as a case of peri-ampullary carcinoma based on physical exam findings and imaging. Ultrasonography (USG) abdomen and magnetic resonance cholangiopancreatography (MRCP) revealed an enlarged common bile duct (CBD) and there was a presence of stricture at the terminal CBD. Endoscopic retrograde cholangiopancreatography (ERCP) showed growth at the ampulla of Vater. An endoscopic ultrasound guided needle core biopsy was obtained. Histopathological examination revealed the case as SRCC at the ampulla of Vater. We present this as an uncommon case of SRCC at the ampulla of Vater.