Carcinoid tumour is an endocrine neoplasia described for the first time in 1888 and rarely observed in the extrahepatic bile ducts. Gallbladder carcinoid tumour was first reported by Joel in 1929. An endoluminal gallbladder lesion, with a bizarre echogenicity, and a mass in the upper pole of the left kidney were found in a 27 year-old man. The patient underwent a cholecystectomy with partial hepatectomy and a polar renal resection. Histological examination revealed a typical gallbladder carcinoid tumour with regional lymph nodal metastasis and a renal cell carcinoma, with morphological and histochemical features of the chromophobe type. This is a distinctive, rare variant, often described in the literature in association with other neoplastic and non-neoplastic diseases. To our knowledge, this is the first report of gallbladder carcinoid tumour with an unexpected aggressive behaviour in a very young patient, with concurrent renal cell carcinoma, chromophobe variant.